MiMeDB: Showing metabocard for L-Leucine (MMDBc0000137)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-10-27 23:37:13 UTC

Update Date

2024-09-28 02:21:36 UTC

Metabolite ID

MMDBc0000137

Metabolite Identification

Common Name

L-Leucine

Description

Leucine (abbreviated as Leu or L) is an Œ±-amino acid that is used in the biosynthesis of proteins. It contains an Œ±-amino group (which is in the protonated ‚àíNH3+ form under biological conditions), an Œ±-carboxylic acid group (which is in the deprotonated ‚àíCOO‚àí form under biological conditions), and a side chain isobutyl group, making it a non-polar aliphatic amino acid. It is essential in humans, meaning the body cannot synthesize it, and it must be obtained from the diet. Human dietary sources are foods that contain protein, such as meats, dairy products, soy products, beans and legumes. In the genetic code, it is encoded by the six codons UUA, UUG, CUU, CUC, CUA, and CUG (Wikipedia ). L-Leucine is a branched-chain amino acid (BCAA). BCAAs are essential amino acids whose carbon structure is marked by a branch point. These three amino acids are critical to human life and are particularly involved in stress, energy, and muscle metabolism. BCAA supplementation as therapy, both oral and intravenous, in human health and disease holds great promise. Despite their structural similarities, the branched amino acids have different metabolic routes, with valine going solely to carbohydrates, leucine solely to fats, and isoleucine to both. The different metabolism accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg, and 16 mg/kg of valine, leucine, and isoleucine respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. Many types of inborn errors of BCAA metabolism exist, and are marked by various abnormalities. The most common form is the maple syrup urine disease, marked by a characteristic urinary odor. Other abnormalities are associated with a wide range of symptoms, such as mental retardation, ataxia, hypoglycemia, spinal muscle atrophy, rash, vomiting, and excessive muscle movement. Most forms of BCAA metabolism errors are corrected by dietary restriction of BCAA and at least one form is correctable by supplementation with 10 mg of biotin daily. BCAA are useful because they are metabolized primarily by muscle. BCAA and other amino acids are frequently fed intravenously (TPN) to malnourished surgical patients and, in some cases, those with severe trauma. BCAA, particularly leucine, stimulate protein synthesis, increase reutilization of amino acids in many organs and reduce protein breakdown. Furthermore, leucine can be an important source of calories, and is superior as fuel to the ubiquitous intravenous glucose (dextrose). Leucine also stimulates insulin release, which in turn stimulates protein synthesis and inhibits protein breakdown. These effects are particularly useful in athletic training. BCAA should also replace the use of steroids as commonly used by weightlifters. Huntington's chorea and anorexic disorders both are characterized by low serum BCAA. These diseases, as well as forms of Parkinson's, may respond to BCAA therapy. BCAA, and particularly leucine, are among the amino acids most essential for muscle health (http://www.dcnutrition.com). L-Leucine can be found in most biofluids, including blood, cerebrospinal fluid (CSF), feces, and sweat, as well as throughout most human tissues. L-Leucine exists in all living species, ranging from bacteria to humans.

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

Synonyms

Value	Source
(2S)-2-Amino-4-methylpentanoic acid	ChEBI
(2S)-alpha-2-Amino-4-methylvaleric acid	ChEBI
(2S)-alpha-Leucine	ChEBI
(S)-(+)-Leucine	ChEBI
(S)-Leucine	ChEBI
2-Amino-4-methylvaleric acid	ChEBI
L	ChEBI
L-Leucin	ChEBI
L-Leuzin	ChEBI
Leu	ChEBI
LEUCINE	ChEBI
(2S)-2-Amino-4-methylpentanoate	Generator
(2S)-a-2-Amino-4-methylvalerate	Generator
(2S)-a-2-Amino-4-methylvaleric acid	Generator
(2S)-alpha-2-Amino-4-methylvalerate	Generator
(2S)-Α-2-amino-4-methylvalerate	Generator
(2S)-Α-2-amino-4-methylvaleric acid	Generator
(2S)-a-Leucine	Generator
(2S)-Α-leucine	Generator
2-Amino-4-methylvalerate	Generator
(S)-2-Amino-4-methylpentanoate	HMDB
(S)-2-Amino-4-methylpentanoic acid	HMDB
(S)-2-Amino-4-methylvalerate	HMDB
(S)-2-Amino-4-methylvaleric acid	HMDB
4-Methyl-L-norvaline	HMDB
L-(+)-Leucine	HMDB
L-a-Aminoisocaproate	HMDB
L-a-Aminoisocaproic acid	HMDB
L-alpha-Aminoisocaproate	HMDB
L-alpha-Aminoisocaproic acid	HMDB
Leucine, L-isomer	HMDB
L-Isomer leucine	HMDB
Leucine, L isomer	HMDB

Molecular Formula

C₆H₁₃NO₂

Average Mass

131.1729

Monoisotopic Mass

131.094628665

IUPAC Name

(2S)-2-amino-4-methylpentanoic acid

Traditional Name

L-leucine

CAS Registry Number

61-90-5

SMILES

CC(C)C[C@H](N)C(O)=O

InChI Identifier

InChI=1S/C6H13NO2/c1-4(2)3-5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t5-/m0/s1

InChI Key

ROHFNLRQFUQHCH-YFKPBYRVSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as leucine and derivatives. Leucine and derivatives are compounds containing leucine or a derivative thereof resulting from reaction of leucine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

Leucine and derivatives

Alternative Parents

Substituents

Leucine or derivatives
Alpha-amino acid
L-alpha-amino acid
Branched fatty acid
Methyl-branched fatty acid
Fatty acid
Fatty acyl
Amino acid
Monocarboxylic acid or derivatives
Carboxylic acid
Organic oxide
Organopnictogen compound
Primary amine
Organooxygen compound
Organonitrogen compound
Primary aliphatic amine
Carbonyl group
Organic oxygen compound
Amine
Organic nitrogen compound
Hydrocarbon derivative
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

proteinogenic amino acid (CHEBI:15603 )
L-alpha-amino acid (CHEBI:15603 )
leucine (CHEBI:15603 )
pyruvate family amino acid (CHEBI:15603 )
Common amino acids (C00123 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Property	Value	Source
Water Solubility	69.8 g/L	ALOGPS
logP	-1.8	ALOGPS
logP	-1.6	ChemAxon
logS	-0.27	ALOGPS
pKa (Strongest Acidic)	2.79	ChemAxon
pKa (Strongest Basic)	9.52	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	63.32 Å²	ChemAxon
Rotatable Bond Count	3	ChemAxon
Refractivity	34.17 m³·mol⁻¹	ChemAxon
Polarizability	14.16 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Spectra

Spectrum Type	Description	Splash Key	Deposition Date	View
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0pb9-0900000000-c0176b3cef05fc597576	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0a4i-0900000000-eb7cee37b9d78694010c	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0a4i-0900000000-7033b5fdcd4216168462	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-0a4i-0900000000-cd1de48a6db61eb4455e	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-05fr-7900000000-e3b993b282ec2115b484	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - EI-B (Non-derivatized)	splash10-0019-9000000000-6e468213b3429cf627bc	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - EI-B (Non-derivatized)	splash10-0a4i-0900000000-ef06e48ca82519977a37	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0pb9-0900000000-c0176b3cef05fc597576	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0a4i-0900000000-eb7cee37b9d78694010c	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0a4i-0900000000-7033b5fdcd4216168462	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0a4i-0900000000-cd1de48a6db61eb4455e	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-QQ (Non-derivatized)	splash10-0udi-1391000000-5183562a14017e557e33	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-05fr-7900000000-e3b993b282ec2115b484	2017-09-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-0006-9000000000-70cdb961a0819be4318a	2016-09-22	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positive	splash10-000i-9100000000-991398731b9d8305622c	2017-10-06	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)	splash10-000i-9100000000-05b5a7a191a32803595e	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)	splash10-0006-9000000000-5302e9c96e75e06e9705	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)	splash10-0006-9000000000-9e70778b46864cd22996	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - EI-B (HITACHI M-80) , Positive	splash10-0019-9000000000-6e468213b3429cf627bc	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-001i-0900000000-4f403c61aaa8a103c049	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-000i-9000000000-d85fcc558423654f45fd	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-000i-9000000000-931937d3bdd49b3ae623	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-0002-0930000000-aa1256c9224fecacf025	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-001i-0900000000-0a72425a86f804d3a16b	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-000i-9000000000-4ed8cafebf4ee4e82e6a	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-000i-9000000000-3ac62b780abd90dd28b1	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-000i-9000000000-eb75eb03a58512ea36df	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative	splash10-001i-0900000000-c0f28e4ebdef67c5b509	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative	splash10-001i-0900000000-0751a9e803a2e7715d1a	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative	splash10-001i-5900000000-8040fc883917239630a7	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative	splash10-016s-9000000000-a7f9e8d43e66dbd02b6f	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive	splash10-001r-7900000000-da119558c426d9c9d3a1	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive	splash10-000i-9000000000-ae8365105ae2a18e0c10	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive	splash10-000f-9000000000-228ee614be6648b37433	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive	splash10-0006-9000000000-d1f3e047af455e156a1e	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive	splash10-0006-9000000000-409dfbdb20719c3afda5	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - CE-ESI-TOF (CE-system connected to 6210 Time-of-Flight MS, Agilent) , Positive	splash10-001i-0900000000-720554d58264a9cfdb67	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positive	splash10-001i-0900000000-90fab591d1f6d6318002	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative	splash10-001i-0900000000-ed843252a559d532dc5e	2012-08-31	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-0019-9500000000-b9f1203176ce0ca7c11c	2016-09-12	View Spectrum
MS	Mass Spectrum (Electron Ionization)	splash10-000l-9000000000-bf752e458f13eed8d7a2	2018-05-25	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 125 MHz, H₂O, experimental)		2012-12-04	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, H₂O, experimental)		2012-12-04	View Spectrum
1D NMR	¹H NMR Spectrum (1D, D₂O, experimental)		2016-10-22	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, H₂O, experimental)		2021-10-10	View Spectrum
2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)		2012-12-05	View Spectrum

Biological Properties

Cellular Locations

Extracellular
Mitochondria

Biospecimen Locations

Adipose Tissue
Adrenal Medulla
Bladder
Blood
Breast Milk
Cerebrospinal Fluid (CSF)
Epidermis
Feces
Fibroblasts
Intestine
Kidney
Neuron
Placenta
Platelet
Prostate
Saliva
Skeletal Muscle
Sweat
Testis
Urine

Tissue Locations

Adipose Tissue
Adrenal Medulla
Bladder
Epidermis
Fibroblasts
Intestine
Kidney
Neuron
Placenta
Platelet
Prostate
Skeletal Muscle
Testis

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species
MMDBp0191971	Branched-chain-amino-acid aminotransferase, cytosolic	Enzyme	P54687	Homo sapiens
MMDBp0192169	Branched-chain-amino-acid aminotransferase, mitochondrial	Enzyme	O15382	Homo sapiens
MMDBp0192238	Probable leucine--tRNA ligase, mitochondrial	Enzyme	Q15031	Homo sapiens
MMDBp0192289	Leucine--tRNA ligase, cytoplasmic	Enzyme	Q9P2J5	Homo sapiens
MMDBp0193522	Large neutral amino acids transporter small subunit 2	Transporter	Q9UHI5	Homo sapiens
MMDBp0193808	4F2 cell-surface antigen heavy chain	Unknown	P08195	Homo sapiens
MMDBp0195767	Leucine carboxyl methyltransferase 2	Unknown	O60294	Homo sapiens
MMDBp0196260	Leucine carboxyl methyltransferase 1	Enzyme	Q9UIC8	Homo sapiens

Human Pathways

Pathways

Name	SMPDB/PathBank
Protein Synthesis: Leucine
Immunometabolism Pathway (Bacterial Activation)
Immunometabolism Pathway (Viral Activation)
Viral Sepsis

Metabolic Reactions

Reactions

Reaction ID	Precursor	Product	Enzyme	Data Source	Reference
MMDBr0001561	Oxoglutaric acid	Ketoleucine	branched chain amino-acid transaminase 2, mitochondrial	VMH; KEGG	30371894
MMDBr0002943	L-Alanine	Primed Glycogenin (Glycogenin-8[1,4-Glc])	glycogenin 1	VMH	10395894 10721716 15489334 8602861 8661012
MMDBr0002944	Primed Glycogenin (Glycogenin-8[1,4-Glc])	L-Alanine	Not Available	VMH	10395894 10721716 15489334 8602861 8661012
MMDBr0004211	Kinetensin	Kinetensin 1-7	Not Available	VMH	1800960
MMDBr0004212	Kinetensin	Kinetensin 1-7	chymase 1, mast cell	VMH	1800960
MMDBr0004213	Kinetensin	Kinetensin 1-8	Not Available	VMH	1800960
MMDBr0004214	Kinetensin	Kinetensin 1-8	carboxypeptidase A1 (pancreatic)	VMH	1800960
MMDBr0004217	Neuromedin N	Neuromedin N (1-4)	Not Available	VMH	1309362
MMDBr0004218	Neuromedin N	Neuromedin N (1-4)	carboxypeptidase A1 (pancreatic)	VMH	1309362
MMDBr0004222	Kinetensin	Kinetensin 1-3	tryptase alpha/beta 1	VMH	1800960
MMDBr0006086	L-Leucine	N-Acetylleucine	Not Available	VMH	30371894
MMDBr0006423	Alanyl-Asparaginyl-Leucine	L-Alanine	Not Available	VMH	30371894
MMDBr0006439	Arginyl-Leucyl-Phenylalanine	L-Arginine	Not Available	VMH	30371894
MMDBr0006474	Cystyl-Leucyl-Threonine	L-Cysteine	Not Available	VMH	30371894
MMDBr0006484	Glutaminyl-Tyrosyl-Leucine	L-Glutamine	Not Available	VMH	30371894
MMDBr0006485	Glutaminyl-Arginyl-Leucine	L-Arginine	Not Available	VMH	30371894
MMDBr0006486	Glutaminyl-Asparaginyl-Leucine	L-Asparagine	Not Available	VMH	30371894
MMDBr0006489	Glutamyl-Leucine	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0006528	Leucyl-Alanyl-Arginine	L-Alanine	Not Available	VMH	30371894
MMDBr0006529	Leucyl-Asparaginyl-Aspartate	L-Asparagine	Not Available	VMH	30371894
MMDBr0006530	Leucyl-Aspartyl-Lysine	L-Aspartic acid	Not Available	VMH	30371894
MMDBr0006531	Leucyl-Leucyl-Tryptophan	L-Leucine	Not Available	VMH	30371894
MMDBr0006532	Leucyl-Proline	L-Leucine	Not Available	VMH	30371894
MMDBr0006533	Leucyl-Prolyl-Arginine	L-Arginine	Not Available	VMH	30371894
MMDBr0006534	Leucyl-Seryl-Tryptophan	L-Leucine	Not Available	VMH	30371894
MMDBr0006535	Leucyl-Tryptophan	L-Leucine	Not Available	VMH	30371894
MMDBr0006536	Leucyl-Tryptophanyl-Arginine	L-Arginine	Not Available	VMH	30371894
MMDBr0006537	Leucyl-Tyrosyl-Tyrosine	L-Leucine	Not Available	VMH	30371894
MMDBr0006538	Leucyl-Valine	L-Leucine	Not Available	VMH	30371894
MMDBr0006539	Lysyl-Arginyl-Leucine	L-Arginine	Not Available	VMH	30371894
MMDBr0006549	Methionyl-Arginyl-Leucine	L-Arginine	Not Available	VMH	30371894
MMDBr0006560	Phenylalanyl-Leucine	L-Leucine	Not Available	VMH	30371894
MMDBr0006561	Phenylalanyl-Leucyl-Aspartate	L-Aspartic acid	Not Available	VMH	30371894
MMDBr0006562	Phenylalanyl-Leucyl-Histidine	L-Histidine	Not Available	VMH	30371894
MMDBr0006571	Phenylalanyl-Tryptophanyl-Leucine	L-Leucine	Not Available	VMH	30371894
MMDBr0006583	Prolyl-Leucyl-Arginine	L-Arginine	Not Available	VMH	30371894
MMDBr0006614	Tryptophanyl-Glutamyl-Leucine	L-Glutamic acid	Not Available	VMH	30371894
MMDBr0006623	Tryptophanyl-Leucyl-Valine	L-Leucine	Not Available	VMH	30371894
MMDBr0006629	Tryptophanyl-Prolyl-Leucine	L-Leucine	Not Available	VMH	30371894
MMDBr0006646	Tyrosyl-Leucyl-Arginine	L-Arginine	Not Available	VMH	30371894
MMDBr0006654	Valyl-Leucyl-Phenylalanine	L-Leucine	Not Available	VMH	30371894
MMDBr0007476	Oxoglutaric acid	Ketoleucine	Not Available	VMH; KEGG	30371894
MMDBr0008207	L-Leucine	Isocaproic acid	Not Available	VMH	30371894
MMDBr0008399	L-Leucine	Ketoleucine	Not Available	VMH	30371894

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
Alzheimer's disease	Decreased	Association	Human Urine	HMDB	17031479
Alzheimer's disease	Slight Increase	Association	Human Cerebrospinal Fluid (CSF)	HMDB	17031479
Alzheimer's disease	Decreased	Association	Human Saliva	HMDB	23857558
Epilepsy	Decreased	Association	Human Blood	HMDB	14992292
Frontotemporal dementia	Slight Decrease	Association	Human Saliva	HMDB	23857558
Fumarase deficiency		Association	Human Blood	HMDB	20549362
Fumarase deficiency		Association	Human Urine	HMDB	20549362
Heart failure	Increased	Association	Human Blood	HMDB	16789974
Leukemia	Slight Decrease	Association	Human Cerebrospinal Fluid (CSF)	HMDB	15911239
Maple syrup urine disease		Association	Human Blood	HMDB	18088602
Maple syrup urine disease	Increased	Association	Human Blood	HMDB	12101068
Phenylketonuria		Association	Human Blood	HMDB	12101068
Schizophrenia		Association	Human Blood	HMDB	2415198
Eosinophilic esophagitis	Slight Decrease	Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Eosinophilic esophagitis		Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Maple syrup urine disease		Association	Human Cerebrospinal Fluid (CSF)	HMDB	Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404
Maple syrup urine disease		Association	Human Blood	HMDB	Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Firmicutes	801	Human feces; Human vaginal secretion; Human ; Human vaginal fluid; Human unknown; Human mucosa; Human saliva; Human stool; Human urine; Human sputum; Human pleural fluid plaque; Pig ; Human subgingival plaque; Human supragingival plaque; Cattle	Microbial culture; Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Eukaryota	Parabasalia	1		Microbial culture
Bacteria	Proteobacteria	389	Human saliva; Human feces; Human sputum; Human ; Human unknown; Pig ; Human pleural fluid plaque; Human dental plaque; Human nasal swab; Human stool; Human feces; pleural fluid plaque; bal; Human subgingival plaque	Microbial culture; Metabolic reconstruction
Eukaryota	Euglenozoa	1		Microbial culture
Bacteria	Synergistetes	8	Human feces	Microbial culture; Metabolic reconstruction
Bacteria	Bacteroidetes	225	Human feces; Human stool; Human saliva; Human ; Human subgingival plaque; Human appendix biopsy; Human gastric fluid; Human urine; Human supragingival plaque	Microbial culture; Metabolic reconstruction; Indirect association between microbiota composition and metabolites measured in host biospecimen
Bacteria	Verrucomicrobia	3	Human feces; Human stool; Human ; Human urine	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria	Actinobacteria	253	Human ; Human feces; Human sputum; Human saliva; Human supragingival plaque; Human subgingival plaque; Human bronchial brush; Human nasopharyngeal swab; Human pleural fluid plaque; Human dental plaque; Human mucosa	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria	Spirochaetes	2	Human feces	Metabolic reconstruction
Archaea	Thaumarchaeota	2		Metabolic reconstruction
Bacteria	Fusobacteria	39	Human feces; Human	Metabolic reconstruction
Archaea	Euryarchaeota	10		Metabolic reconstruction
Bacteria	Candidatus melainabacteria	2	Human feces	Metabolic reconstruction
Bacteria	Tenericutes	8	Human feces	Metabolic reconstruction
Bacteria	Planctomycetes	2	Human feces	Metabolic reconstruction
Archaea	Crenarchaeota	2		Metabolic reconstruction
Eukaryota/Fungi	Ascomycota	10	Human feces; Human saliva	Unknown
Bacteria	Aquificae	1
Archaea/Archaea	Euryarchaeota	5		Metabolic reconstruction
Bacteria	nah	1
Bacteria	Thermotogae	1

Exposure Sources

Source Type	Activity	Data Source	Reference
Drugs	Antimicrobial	U.S. Environmental Protection Agency	CompTox
Cosmetics	Not Available	U.S. Environmental Protection Agency	CompTox
Domestics	Not Available	U.S. Environmental Protection Agency	CompTox
Domestics	Not Available	U.S. Environmental Protection Agency	CompTox
Cosmetics	Not Available	U.S. Environmental Protection Agency	CompTox

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Std	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Feces	Detected but not Quantified									HMDB
Human Urine	Detected and Quantified	0.91	0.11			umol/mmol creatinine	Adult (>18 years old)	Both	Alzheimer's disease	HMDB	17031479
Human Cerebrospinal fluid (csf)	Detected and Quantified	11.5	1.5			uM	Adult (>18 years old)	Both	Alzheimer's disease	HMDB	17031479
Human Saliva	Detected and Quantified	2.73	0.76			uM	Adult (>18 years old)	Male	Alzheimer's disease	HMDB	23857558
Human Blood	Detected and Quantified			15	113	uM	Children (1-13 years old)	Both	Branched-chain Keto Acid Dehydrogenase Kinase Deficiency	HMDB	22956686
Human Blood	Detected and Quantified			18	46	uM	Adolescent (13-18 years old)	Female	Branched-chain Keto Acid Dehydrogenase Kinase Deficiency	HMDB	22956686
Human Blood	Detected and Quantified			20	46	uM	Adult (>18 years old)	Female	Branched-chain Keto Acid Dehydrogenase Kinase Deficiency	HMDB	22956686
Human Blood	Detected and Quantified	686.117				uM	Infant (0-1 year old)	Male	Dihydrolipoamide Dehydrogenase Deficiency	HMDB	6688766
Human Blood	Detected and Quantified	67.5	15.5			uM	Adult (>18 years old)	Female	Early preeclampsia	HMDB	22494326
Human Blood	Detected and Quantified	103.0		97.8	108.0	uM	Adult (>18 years old)	Both	Epilepsy	HMDB	14992292
Human Blood	Detected and Quantified	95.3		89.3	100.0	uM	Adult (>18 years old)	Both	Epilepsy	HMDB	14992292
Human Cerebrospinal fluid (csf)	Detected and Quantified	8.2				uM	Children (1-13 years old)	Female	Epilepsy, early-onset, vitamin B6-dependent	HMDB	27912044
Human Cerebrospinal fluid (csf)	Detected and Quantified	26.7				uM	Newborn (0-30 days old)	Female	Epilepsy, early-onset, vitamin B6-dependent	HMDB	27912044
Human Saliva	Detected and Quantified	3.74	0.65			uM	Adult (>18 years old)	Male	Frontotemporal lobe dementia	HMDB	23857558
Human Blood	Detected and Quantified	66				uM	Infant (0-1 year old)	Male	Fumaric aciduria	HMDB	20549362
Human Urine	Detected and Quantified			53.900	17.750	umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Fumaric aciduria	HMDB	20549362
Human Blood	Detected and Quantified	273.0	10.0			uM	Adult (>18 years old)	Both	Heart failure	HMDB	16789974
Human Blood	Detected and Quantified	114.5	98.5			uM	Adult (>18 years old)	Female	Late-onset preeclampsia	HMDB	23159745
Human Cerebrospinal fluid (csf)	Detected and Quantified	13.1	5.6			uM	Children (1-13 years old)	Not Specified	Leukemia	HMDB	15911239
Human Cerebrospinal fluid (csf)	Detected and Quantified	16.5	7.0			uM	Children (1-13 years old)	Not Specified	Leukemia	HMDB	15911239
Human Saliva	Detected and Quantified	5.66	3.83			uM	Adult (>18 years old)	Both	Lewy body disease	HMDB	23857558
Human Blood	Detected and Quantified	67.5		15	76	uM	Children (1-13 years old)	Male	Lipoyltransferase 1 Deficiency	HMDB	24341803
Human Blood	Detected and Quantified	365.9	287.9			uM	Children (1 - 13 years old)	Not Specified	Maple syrup urine disease	HMDB	18088602
Human Blood	Detected and Quantified			643.0	667.0	uM	Newborn (0-30 days old)	Both	Maple syrup urine disease (MSUD)	HMDB	12101068
Human Blood	Detected and Quantified			66.0	161.0	uM	Newborn (0-30 days old)	Both	Phenylketonuria	HMDB	12101068
Human Blood	Detected and Quantified	60.0	46.9			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	23313728
Human Blood	Detected and Quantified	69.55	16.39			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	24704061
Human Blood	Detected and Quantified	71.6	40.6			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	23535240
Human Blood	Detected and Quantified	87.1	61.9			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	23159745
Human Blood	Detected and Quantified	96.9	74.9			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	22494326
Human Blood	Detected and Quantified	141	26			uM	Adult (>18 years old)	Female	Schizophrenia	HMDB	2415198
Human Blood	Detected and Quantified	171	30			uM	Adult (>18 years old)	Male	Schizophrenia	HMDB	2415198
Human Urine	Detected and Quantified	1.2				umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	21388201
Human Urine	Detected and Quantified	3.5				umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	19309105
Human Feces	Detected and Quantified	7298				nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	HMDB	26416813
Human Feces	Detected and Quantified	7601				nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	HMDB	26416813
Human Feces	Detected and Quantified	8209				nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	HMDB	26416813
Human Sweat	Detected and Quantified	< 10				uM	Adult (60 years old)	Male	Normal	HMDB	22194922
Human Sweat	Detected and Quantified	< 10				uM	Adult (40 years old)	Male	Normal	HMDB	22194922
Human Urine	Detected and Quantified			0.33	13.41	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	32340350
Human Urine	Detected and Quantified			0.45	17.10	umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	HMDB	25536744
Human Urine	Detected and Quantified	0.6	0.2			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	3168222
Human Urine	Detected and Quantified	0.6	0.3			umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	HMDB	3168222
Human Urine	Detected and Quantified	0.6	0.3			umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	HMDB	3168222
Human Urine	Detected and Quantified			1.15	19.93	umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	HMDB	25536744
Human Urine	Detected and Quantified			1.4	2.3	umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	27012787
Human Urine	Detected and Quantified			1.4	2.3	umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	27012787
Human Cerebrospinal fluid (csf)	Detected and Quantified			11.0	18.7	uM	Children (1-13 years old)	Not Specified	Normal	HMDB	27912044
Human Cerebrospinal fluid (csf)	Detected and Quantified	11.2	3.5			uM	Adult (>18 years old)	Not Specified	Normal	HMDB	6198473
Human Saliva	Detected and Quantified	11.3	7.6			uM	Adult (>18 years old)	Male	Normal	HMDB	21164041
Human Blood	Detected and Quantified	120	11			uM	Adult (>18 years old)	Female	Normal	HMDB	2415198
Human Blood	Detected and Quantified	123		98	148	uM	Adult (>18 years old)	Both	Normal	HMDB	12297216
Human Blood	Detected and Quantified	127.4		100.5	150.2	uM	Children (1-13 uears old)	Both	Normal	HMDB	27294788
Human Urine	Detected and Quantified			13	40	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	31506554
Human Cerebrospinal fluid (csf)	Detected and Quantified			14.1	22.9	uM	Newborn (0-30 days old)	Not Specified	Normal	HMDB	27912044
Human Cerebrospinal fluid (csf)	Detected and Quantified	14.5	3.7			uM	Adult (>18 years old)	Not Specified	Normal	HMDB	15911239
Human Blood	Detected and Quantified	150.1	27.7			uM	Adult (>18 years old)	Both	Normal	HMDB	28278231
Human Blood	Detected and Quantified	156		134	183	uM	Newborn (0-30 days old)		Normal	HMDB	25754623
Human Blood	Detected and Quantified	158.3	37.6			uM	Children (1 - 13 years old)	Not Specified	Normal	HMDB	18088602
Human Blood	Detected and Quantified	159	23			uM	Adult (>18 years old)	Male	Normal	HMDB	2415198
Human Cerebrospinal fluid (csf)	Detected and Quantified	16	9			uM	Adult (>18 years old)	Both	Normal	HMDB	18502700
Human Blood	Detected and Quantified	168		134	216	uM	Infant (0-1 year old)		Normal	HMDB	25754623
Human Feces	Detected and Quantified	1780	1160			nmol/g wet feces	Adult (>18 years old)	Both	Normal	HMDB	27624970
Human Cerebrospinal fluid (csf)	Detected and Quantified	18.6	4.1			uM	Adult (>18 years old)	Not Specified	Normal	HMDB	6198473
Human Urine	Detected and Quantified	2.8		1.6	5.4	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Feces	Detected and Quantified	2014.898	1413.402			nmol/g wet feces	Not Specified	Not Specified	Normal	HMDB	20669995
Human Feces	Detected and Quantified	2076¬†	¬†837			nmol/g wet feces	Adult (>18 years old)	Female	Normal	HMDB	23997757
Human Breast milk	Detected and Quantified	24.4	6.3			uM	Adult (>18 years old)	Female	Normal	HMDB	24027187
Human Blood	Detected and Quantified	251	5			uM	Adult (>18 years old)	Both	Normal	HMDB	16789974
Human Urine	Detected and Quantified	28.27	11.31			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	19468821
Human Urine	Detected and Quantified	3.0		1.6	6.0	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified			3.166	45.450	umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	HMDB	20549362
Human Feces	Detected and Quantified	300	290			nmol/g wet feces	Adult (>18 years old)	Both	Normal	HMDB	27624970
Human Urine	Detected and Quantified	4.030	4.10			umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	HMDB	25536744
Human Saliva	Detected and Quantified	4.2	3.6			uM	Adult (>18 years old)	Male	Normal	HMDB	21164041
Human Urine	Detected and Quantified	44.09	22.61			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	19468821
Human Urine	Detected and Quantified	44.09	24.87			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	19468821
Human Blood	Detected and Quantified			48	160	uM	Infant (0-1 year old)	Not Specified	Normal	HMDB	20549362
Human Blood	Detected and Quantified			49	216	uM	Children (1-13 years old)	Not Specified	Normal	HMDB	22956686
Human Urine	Detected and Quantified	5.4	2.86			umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	HMDB	32340350
Human Feces	Detected and Quantified	50.773	61.369			nmol/g wet feces	Not Specified	Not Specified	Normal	HMDB	19573517
Human Urine	Detected and Quantified	6.26	2.87			umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	HMDB	32340350
Human Saliva	Detected and Quantified	6.7	5.8			uM	Adult (>18 years old)	Male	Normal	HMDB	21164041
Human Blood	Detected and Quantified			64	164	uM	Children (1-13 years old)	Not Specified	Normal	HMDB	22956686
Human Blood	Detected and Quantified			68	168	uM	Infant (0-1 year old)	Not Specified	Normal	HMDB	24341803
Human Saliva	Detected and Quantified	7.24	5.62			uM	Adult (>18 years old)	Female	Normal	HMDB	23857558
Human Urine	Detected and Quantified	7.37	5.63			umol/mmol creatinine	Children (0-2 years old)	Male	Normal	HMDB	25536744
Human Urine	Detected and Quantified	7.46	5.15			umol/mmol creatinine	Children (0-2 years old)	Female	Normal	HMDB	25536744
Human Urine	Detected and Quantified	7.58	4.10			umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	2026685
Human Blood	Detected and Quantified			72	201	uM	Adult (>18 years old)	Not Specified	Normal	HMDB	22956686
Human Blood	Detected and Quantified			76	168	uM	Adolescent (13-18 years old)	Not Specified	Normal	HMDB	22956686
Human Saliva	Detected and Quantified	8.1	4.1			uM	Adult (>18 years old)	Male	Normal	HMDB	21164041
Human Urine	Detected and Quantified	8.29	6.53			umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	HMDB	25536744
Human Blood	Detected and Quantified	98.7	11.5			uM	Adult (>18 years old)	Both	Normal	HMDB	21359215
Human Urine	Detected and Quantified	6.3164	4.2963			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	8.333	7.338			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Cerebrospinal fluid (csf)	Detected and Quantified	892				uM	Children (1-13 years old)	Female	Maple syrup urine disease	HMDB	Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404
Human Blood	Detected and Quantified	1694				uM	Children (1-13 years old)	Female	Maple syrup urine disease	HMDB	Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404
Human Urine	Detected and Quantified	7.547	4.945			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	8.0076	3.0528			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified			1.24	8.37	umol/mmol creatinine	Adolescent (13 - 21 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified			1.24	8.37	umol/mmol creatinine	Adult (>21 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Cerebrospinal fluid (csf)	Detected and Quantified			1.4	23.6	uM	Adolescent (>11 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified			2.04	12.44	umol/mmol creatinine	Children (4 - 13 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified			2.04	13.23	umol/mmol creatinine	Children (1 - 2 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified			2.04	19.67	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified			2.83	12.44	umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified			2.83	14.02	umol/mmol creatinine	Children (2 - 4 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified			2.83	9.27	umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Cerebrospinal fluid (csf)	Detected and Quantified			3.3	21.3	uM	Children (0 - 10 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Blood	Detected and Quantified			40	180	uM	Children (3 months - 6 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Blood	Detected and Quantified			43	165	uM	Infant (1 - 3 months old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Blood	Detected and Quantified			55	174	uM	Children (6 - 18 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Blood	Detected and Quantified			55	183	uM	Newborn (0-30 days old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified			6.10	19.07	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	David F. Putnam Composition and Concentrative Properties of Human Urine. NASA Contractor Report. July 1971
Human Cerebrospinal fluid (csf)	Detected and Quantified	10.1	2.1			uM	Adult (>18 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Blood	Detected and Quantified	134.0	20.0			uM	Children (1-13 years old)	Male	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Blood	Detected and Quantified	157.0	30.0			uM	Adult (>18 years old)	Male	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Blood	Detected and Quantified	160.0	27.0			uM	Adult (>18 years old)	Female	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	2.5	1.0			umol/mmol creatinine	Children (1-13 years old)	Male	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	2.5	1.3			umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	3.0	1.5			umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	6.67		0	13.33	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Blood	Detected and Quantified	70.0	25.0			uM	Newborn (0-30 days old)	Not Specified	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Saliva	Detected and Quantified	16.0	21.5			uM	Adult (>18 years old)	Male	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	2.79	1.21			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	3.80	2.10			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	6.61	4.84			uM	Adult (>18 years old)	Both	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	53.50	32.95			uM	Adult (>18 years old)	Both	Normal	HMDB	https://doi.org/10.1007/s11306-015-0840-5
Human Blood	Detected and Quantified			70.00	180.0	uM	Adult (>18 years old)	Both	Normal	HMDB	Molecular You (https://molecularyou.com)
Human Urine	Detected and Quantified	<22.62				umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<5.77				umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<7.013				umol/mmol creatinine	Adolescent (13-18 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified			1.36	11.31	umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified			1.47	8.26	umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified			1.70	18.89	umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Adipose tissue	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Adrenal medulla	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Bladder	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Epidermis	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Fibroblasts	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Intestine	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Kidney	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Neuron	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Placenta	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Platelet	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Prostate	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Skeletal muscle	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Testis	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	20300169
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Not Specified	Normal	HMDB	22727327
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Not Specified	Normal	HMDB	12097436
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	21190195
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	17269711
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	18052033
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	18085514
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	19006102
Human Feces	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	19573517
Human Feces	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	20669995
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	21761941
Human Feces	Detected but not Quantified						Infant (0-1 year old)	Both	Normal	HMDB	22411374
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	23940645
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	24029555
Human Feces	Detected but not Quantified						Infant (0-1 year old)	Both	Normal	HMDB	24375085
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	25670064
Human Feces	Detected but not Quantified						Adult (>18 years old)	Female	Normal	HMDB	26709303
Human Feces	Detected but not Quantified						Not Specified	Both	Normal	HMDB	25486321
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	17269711
Human Feces	Detected but not Quantified						Children (1-13 years old)	Both	Normal	HMDB	24130822
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	25598765
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27065191
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27107423
Human Feces	Detected but not Quantified						Children (6 - 18 years old)	Not Specified	Normal	HMDB	27609529
Human Sweat	Detected but not Quantified						Adult	Both	Normal	HMDB	26755145
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27543620
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	25037050
Human Feces	Detected but not Quantified						Adult (>18 years old)	Not Specified	Normal	HMDB	25985090
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	28587349
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	29808030
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	32966057
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	32966057

External Links

HMDB ID

HMDB0000687

DrugBank ID

DB00149

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Wikipedia Link

METLIN ID

PubChem Compound

6106

PDB ID

Not Available

ChEBI ID

15603

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

Lichtenstein AH, Hachey DL, Millar JS, Jenner JL, Booth L, Ordovas J, Schaefer EJ: Measurement of human apolipoprotein B-48 and B-100 kinetics in triglyceride-rich lipoproteins using [5,5,5-2H3]leucine. J Lipid Res. 1992 Jun;33(6):907-14. [PubMed:1512514 ]
Hagenfeldt L, Bjerkenstedt L, Edman G, Sedvall G, Wiesel FA: Amino acids in plasma and CSF and monoamine metabolites in CSF: interrelationship in healthy subjects. J Neurochem. 1984 Mar;42(3):833-7. doi: 10.1111/j.1471-4159.1984.tb02756.x. [PubMed:6198473 ]
Nicholson JK, O'Flynn MP, Sadler PJ, Macleod AF, Juul SM, Sonksen PH: Proton-nuclear-magnetic-resonance studies of serum, plasma and urine from fasting normal and diabetic subjects. Biochem J. 1984 Jan 15;217(2):365-75. doi: 10.1042/bj2170365. [PubMed:6696735 ]
Sakamoto M, Nakao K, Yoshimasa T, Ikeda Y, Suda M, Takasu K, Shimbo S, Yanaihara N, Imura H: Occurrence of methionine-enkephalin-Arg6-Gly7-Leu8 with methionine-enkephalin, leucine-enkephalin and methionine-enkephalin-Arg6-Phe7 in human gastric antrum. J Clin Endocrinol Metab. 1983 Jan;56(1):202-4. doi: 10.1210/jcem-56-1-202. [PubMed:6847871 ]
Yoshimasa T, Nakao K, Ohtsuki H, Li S, Imura H: Methionine-enkephalin and leucine-enkephalin in human sympathoadrenal system and pheochromocytoma. J Clin Invest. 1982 Mar;69(3):643-50. doi: 10.1172/jci110491. [PubMed:7061706 ]
Jansson T, Scholtbach V, Powell TL: Placental transport of leucine and lysine is reduced in intrauterine growth restriction. Pediatr Res. 1998 Oct;44(4):532-7. doi: 10.1203/00006450-199810000-00011. [PubMed:9773842 ]
Mero A: Leucine supplementation and intensive training. Sports Med. 1999 Jun;27(6):347-58. doi: 10.2165/00007256-199927060-00001. [PubMed:10418071 ]
Iannoli P, Miller JH, Wang HT, Bode B, Souba WW, Avissar NE, Sax HC: Characterization of L-leucine transport system in brush border membranes from human and rabbit small intestine. Metabolism. 1999 Nov;48(11):1432-6. doi: 10.1016/s0026-0495(99)90155-5. [PubMed:10582553 ]
Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. doi: 10.1016/s1570-0232(02)00283-0. [PubMed:12101068 ]
Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. doi: 10.1016/s0899-9007(02)00780-3. [PubMed:12297216 ]
Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. doi: 10.1023/b:nere.0000010461.34920.0c. [PubMed:14992292 ]
Etzel MR: Manufacture and use of dairy protein fractions. J Nutr. 2004 Apr;134(4):996S-1002S. doi: 10.1093/jn/134.4.996S. [PubMed:15051860 ]
Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. doi: 10.1016/j.ejca.2005.02.013. Epub 2005 Apr 14. [PubMed:15911239 ]
Yudkoff M, Daikhin Y, Nissim I, Horyn O, Luhovyy B, Luhovyy B, Lazarow A, Nissim I: Brain amino acid requirements and toxicity: the example of leucine. J Nutr. 2005 Jun;135(6 Suppl):1531S-8S. doi: 10.1093/jn/135.6.1531S. [PubMed:15930465 ]
Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
Wilkinson DJ, Hossain T, Hill DS, Phillips BE, Crossland H, Williams J, Loughna P, Churchward-Venne TA, Breen L, Phillips SM, Etheridge T, Rathmacher JA, Smith K, Szewczyk NJ, Atherton PJ: Effects of leucine and its metabolite beta-hydroxy-beta-methylbutyrate on human skeletal muscle protein metabolism. J Physiol. 2013 Jun 1;591(11):2911-23. doi: 10.1113/jphysiol.2013.253203. Epub 2013 Apr 3. [PubMed:23551944 ]
Lynch CJ, Adams SH: Branched-chain amino acids in metabolic signalling and insulin resistance. Nat Rev Endocrinol. 2014 Dec;10(12):723-36. doi: 10.1038/nrendo.2014.171. Epub 2014 Oct 7. [PubMed:25287287 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Showing metabocard for L-Leucine (MMDBc0000137)

MOL for #<Metabolite:0x00007fdb486f0538>

3D MOL for #<Metabolite:0x00007fdb486f0538>

3D SDF for #<Metabolite:0x00007fdb486f0538>

3D-SDF for #<Metabolite:0x00007fdb486f0538>

PDB for #<Metabolite:0x00007fdb486f0538>

3D PDB for #<Metabolite:0x00007fdb486f0538>

SMILES for #<Metabolite:0x00007fdb486f0538>

INCHI for #<Metabolite:0x00007fdb486f0538>

Structure for #<Metabolite:0x00007fdb486f0538>

3D Structure for #<Metabolite:0x00007fdb486f0538>