MiMeDB: Showing metabocard for (R)-Carnitine (MMDBc0033059)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2021-11-19 04:32:58 UTC

Update Date

2024-09-29 10:01:07 UTC

Metabolite ID

MMDBc0033059

Metabolite Identification

Common Name

(R)-Carnitine

Description

Carnitine is a non-essential amino acid and a quaternary ammonium compound. Carnitine is also classified as an alcohol (specifically, a trimethylated carboxy-alcohol). Carnitine exists as one of two stereoisomers (the two enantiomers D-carnitine and L-carnitine. Both are biologically active, but only L-carnitine naturally occurs in animals, and D-carnitine is toxic as it inhibits the activity of the L-form. Carnitine is involved in the metabolism in most mammals, plants, and some bacteria. Carnitine plays a key role in lipid metabolism and beta-oxidation. It is used to transport long-chain fatty acids into the mitochondria to be oxidized for energy production. This is done by forming a long chain acetylcarnitine esters which are then transported by carnitine palmitoyltransferase I and carnitine palmitoyltransferase II. Carnitine also participates in removing products of metabolism from cells. Given its key metabolic roles, carnitine is concentrated in skeletal and cardiac muscle as well as other tissues that metabolize fatty acids as an energy source. A normal 70 kilogram person typically produces 11-34 mg of carnitine per day. Adults eating mixed diets of red meat and other animal products ingest 60-180 mg of carnitine per day, while vegans consume about 10-12 mg per day. Most carnitine obtained from the diet is absorbed in the small intestine before entering the blood.[3] The total body content of carnitine is about 20 grams in a person weighing 70 kilograms, with nearly all of it contained within skeletal muscle cells. Carnitine is so important in providing energy to muscles (including the heart) that some researchers are now recommending carnitine supplements in the diet, particularly for people who do not consume much red meat (the main food source for carnitine). Carnitine has been described as a vitamin, an amino acid, or a metabimin (i.e. an essential metabolite). Like the B vitamins, carnitine contains nitrogen and is very soluble in water. However, most animals, including humans, make their own carnitine; thus, carnitine cannot be considered to be a vitamin. In certain circumstances, such as methionine deficiency, lysine deficiency, vitamin C deficiency or kidney dialysis, carnitine shortages can develop. Under these conditions, carnitine must be absorbed from food, and for this reason, it is sometimes referred to as a "metabimin" or a conditionally essential metabolite. In humans, about 25% of carnitine is synthesized in the liver, kidney, and brain from lysine and methionine. Most of the carnitine in the body comes from dietary sources such as red meat and dairy products. Inborn errors of carnitine metabolism such as Reye‚Äôs syndrome can lead to brain deterioration gradually worsening muscle weakness, Duchenne-like muscular dystrophy, and extreme muscle weakness with fat accumulation in muscles. Carnitine is an essential nutrient for pre-term babies and individuals who are unable to eat a normal diet (e.g. non-ketotic hypoglycemics, kidney dialysis patients) (PMID: 115309 ). In conditions such as kwashiorkor, cirrhosis, and heart muscle disease (cardiomyopathy) as well as in inborn errors of metabolism such as type IV hyperlipidemia and propionic aciduria, carnitine is essential to life and carnitine supplements are critically important. Carnitine therapy may also be useful in a wide variety of clinical conditions. Carnitine supplementation has improved some patients who have angina secondary to coronary artery disease. Carnitine supplements may also be useful in many forms of metabolic liver diseases and heart muscle disease. Hearts undergoing severe arrhythmia quickly deplete their stores of carnitine. Athletes, particularly in Europe, have used carnitine supplements for improved endurance. Carnitine may improve muscle building by improving fat utilization and may even be useful in treating obesity. Carnitine may be of value in treating pregnant women, hypothyroid individuals, and male infertility due to the low motility of sperm. Carnitine deficiency is noted in abnormal liver function, renal dialysis patients, and severe to moderate muscular weakness with associated anorexia. Carnitine is a biomarker for the consumption of meat. In fact, carnitine was first extracted from meat extracts in 1905, leading to its name from Latin, "caro/carnis" or flesh.

Structure

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

COMPND    HMDB0000062
HETATM    1  C1  UNL     1      -2.045  -1.115   0.460  1.00  0.00           C  
HETATM    2  N1  UNL     1      -1.493   0.086  -0.133  1.00  0.00           N1+
HETATM    3  C2  UNL     1      -1.537   1.160   0.811  1.00  0.00           C  
HETATM    4  C3  UNL     1      -2.389   0.459  -1.238  1.00  0.00           C  
HETATM    5  C4  UNL     1      -0.220  -0.193  -0.694  1.00  0.00           C  
HETATM    6  C5  UNL     1       0.932  -0.184   0.255  1.00  0.00           C  
HETATM    7  O1  UNL     1       1.139   1.013   0.899  1.00  0.00           O  
HETATM    8  C6  UNL     1       2.196  -0.448  -0.574  1.00  0.00           C  
HETATM    9  C7  UNL     1       3.368  -0.446   0.313  1.00  0.00           C  
HETATM   10  O2  UNL     1       3.622  -1.470   1.009  1.00  0.00           O  
HETATM   11  O3  UNL     1       4.199   0.647   0.408  1.00  0.00           O  
HETATM   12  H1  UNL     1      -2.692  -0.848   1.332  1.00  0.00           H  
HETATM   13  H2  UNL     1      -2.668  -1.648  -0.287  1.00  0.00           H  
HETATM   14  H3  UNL     1      -1.206  -1.747   0.798  1.00  0.00           H  
HETATM   15  H4  UNL     1      -1.005   2.059   0.450  1.00  0.00           H  
HETATM   16  H5  UNL     1      -1.259   0.867   1.840  1.00  0.00           H  
HETATM   17  H6  UNL     1      -2.620   1.480   0.884  1.00  0.00           H  
HETATM   18  H7  UNL     1      -3.121   1.216  -0.898  1.00  0.00           H  
HETATM   19  H8  UNL     1      -1.797   0.926  -2.069  1.00  0.00           H  
HETATM   20  H9  UNL     1      -2.958  -0.412  -1.612  1.00  0.00           H  
HETATM   21  H10 UNL     1      -0.256  -1.197  -1.170  1.00  0.00           H  
HETATM   22  H11 UNL     1      -0.021   0.526  -1.525  1.00  0.00           H  
HETATM   23  H12 UNL     1       0.858  -1.017   0.968  1.00  0.00           H  
HETATM   24  H13 UNL     1       1.398   0.820   1.834  1.00  0.00           H  
HETATM   25  H14 UNL     1       2.104  -1.432  -1.077  1.00  0.00           H  
HETATM   26  H15 UNL     1       2.282   0.370  -1.328  1.00  0.00           H  
HETATM   27  H16 UNL     1       5.190   0.528   0.342  1.00  0.00           H  
CONECT    1    2   12   13   14
CONECT    2    3    4    5
CONECT    3   15   16   17
CONECT    4   18   19   20
CONECT    5    6   21   22
CONECT    6    7    8   23
CONECT    7   24
CONECT    8    9   25   26
CONECT    9   10   10   11
CONECT   11   27
END

Synonyms

Value	Source
(-)-Carnitine	ChEBI
(-)-L-Carnitine	ChEBI
3-Carboxy-2-hydroxy-N,N,N-trimethyl-1-propanaminium hydroxide, inner salt	ChEBI
Carnicor	ChEBI
Carnitene	ChEBI
Carnitine	ChEBI
Carnitor	ChEBI
Karnitin	ChEBI
Levocarnitine	ChEBI
Vitamin BT	ChEBI
L-gamma-Trimethyl-beta-hydroxybutyrobetaine	Kegg
(R)-Carnitine	Kegg
L-g-Trimethyl-b-hydroxybutyrobetaine	Generator
L-Γ-trimethyl-β-hydroxybutyrobetaine	Generator
(-)-(R)-3-Hydroxy-4-(trimethylammonio)butyrate	HMDB
(R)-(3-Carboxy-2-hydroxypropyl)trimethylammonium hydroxide	HMDB
(S)-Carnitine	HMDB
1-Carnitine	HMDB
3-Carboxy-2-hydroxy-N,N,N-trimethyl-1-propanaminium	HMDB
3-Hydroxy-4-trimethylammoniobutanoate	HMDB
3-Hydroxy-4-trimethylammoniobutanoic acid	HMDB
Bicarnesine	HMDB
Carniking	HMDB
Carniking 50	HMDB
Carnilean	HMDB
Carnipass	HMDB
Carnipass 20	HMDB
D-Carnitine	HMDB
delta-Carnitine	HMDB
DL-Carnitine	HMDB
gamma-Trimethyl-ammonium-beta-hydroxybutirate	HMDB
gamma-Trimethyl-beta-hydroxybutyrobetaine	HMDB
gamma-Trimethyl-hydroxybutyrobetaine	HMDB
L-(-)-Carnitine	HMDB
Levocarnitina	HMDB
Levocarnitinum	HMDB
R-(-)-3-Hydroxy-4-trimethylaminobutyrate	HMDB
L Carnitine	HMDB
L-Carnitine	ChEBI

Molecular Formula

C₇H₁₅NO₃

Average Mass

161.1989

Monoisotopic Mass

161.105193351

IUPAC Name

(3R)-3-hydroxy-4-(trimethylazaniumyl)butanoate

Traditional Name

L-carnitine

CAS Registry Number

541-15-1

SMILES

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

InChI Identifier

InChI=1S/C7H15NO3/c1-8(2,3)5-6(9)4-7(10)11/h6,9H,4-5H2,1-3H3/t6-/m1/s1

InChI Key

PHIQHXFUZVPYII-ZCFIWIBFSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as carnitines. These are organic compounds containing the quaternary ammonium compound carnitine.

Kingdom

Organic compounds

Super Class

Organic nitrogen compounds

Class

Organonitrogen compounds

Sub Class

Quaternary ammonium salts

Direct Parent

Carnitines

Alternative Parents

Substituents

Carnitine
Beta-hydroxy acid
Short-chain hydroxy acid
Fatty acid
Hydroxy acid
Tetraalkylammonium salt
1,2-aminoalcohol
Carboxylic acid salt
Secondary alcohol
Carboxylic acid derivative
Carboxylic acid
Monocarboxylic acid or derivatives
Organic oxygen compound
Organooxygen compound
Organic zwitterion
Organic salt
Hydrocarbon derivative
Organic oxide
Carbonyl group
Organopnictogen compound
Amine
Alcohol
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

carnitine (CHEBI:16347 )

Functional Ontology

Not Available

Physical Properties

State

Solid

Predicted Properties

Property	Value	Source
Water Solubility	5.33 g/L	ALOGPS
logP	-2.9	ALOGPS
logP	-4.9	ChemAxon
logS	-1.6	ALOGPS
pKa (Strongest Acidic)	4.2	ChemAxon
pKa (Strongest Basic)	-3.6	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	1	ChemAxon
Polar Surface Area	60.36 Å²	ChemAxon
Rotatable Bond Count	4	ChemAxon
Refractivity	63.49 m³·mol⁻¹	ChemAxon
Polarizability	16.93 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
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-00di-9100000000-1b25dacb04c3ed5be8d0	2016-09-22	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positive	splash10-00di-9100000000-c15eaa190d1e28a4839a	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 (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	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 (TMS_2_1) - 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
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-03di-0900000000-9b579c570aab7c7d3a21	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-0ik9-0900000000-e37d3def2de1af5bd2f5	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-0ue9-8900000000-cf37f0a721cd52d86e8e	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-0r0c-9200000000-a11a4872036f3c33d25a	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , positive	splash10-0a4l-9000000000-2402e260a330ff1772a1	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT , positive	splash10-0w29-6900000000-d0136248acdd706e650b	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF , positive	splash10-0ik9-2900000000-61732bb10307f2b183ca	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF , positive	splash10-0zg0-9400000000-15c3db3324f1d46d3c7c	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF , positive	splash10-0ik9-2900000000-ee79f10dce9fc09246ec	2017-09-14	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-01ox-0900000000-3bffd5143cf8b072299e	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-00kf-0900000000-e68f553bcb15d3ef5b47	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-00di-9200000000-c5b43723951af6da48cf	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-03di-1900000000-dd1b1a023937cdcb11af	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-114i-3900000000-9f24d3bc535736149e2a	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0a4l-9000000000-632ef17d9dde38586990	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-03di-3900000000-97af6636a917c0edea61	2021-09-24	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-01pc-9300000000-543e21cb6b9785149e31	2021-09-24	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-08fr-9000000000-9128d6fe96d9c7276695	2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, D₂O, experimental)		2016-10-22	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Endoplasmic reticulum
Extracellular
Mitochondria
Peroxisome

Biospecimen Locations

Adipose Tissue
Bladder
Blood
Brain
Breast Milk
Cerebrospinal Fluid (CSF)
Erythrocyte
Feces
Fibroblasts
Heart
Intestine
Kidney
Liver
Lung
Neuron
Placenta
Platelet
Prostate
Saliva
Semen
Skeletal Muscle
Testis
Urine

Tissue Locations

Adipose Tissue
Bladder
Brain
Erythrocyte
Fibroblasts
Heart
Intestine
Kidney
Liver
Lung
Neuron
Placenta
Platelet
Prostate
Semen
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
MMDBp0191704	Carnitine O-acetyltransferase	Enzyme	P43155	Homo sapiens
MMDBp0191852	Xanthine dehydrogenase/oxidase	Unknown	P47989	Homo sapiens
MMDBp0191952	Myeloperoxidase	Unknown	P05164	Homo sapiens
MMDBp0191977	Peroxisomal carnitine O-octanoyltransferase	Enzyme	Q9UKG9	Homo sapiens
MMDBp0191978	Carnitine O-palmitoyltransferase 1, muscle isoform	Enzyme	Q92523	Homo sapiens
MMDBp0191979	Carnitine O-palmitoyltransferase 1, liver isoform	Enzyme	P50416	Homo sapiens
MMDBp0191981	Carnitine O-palmitoyltransferase 2, mitochondrial	Enzyme	P23786	Homo sapiens
MMDBp0191982	Carnitine O-palmitoyltransferase 1, brain isoform	Enzyme	Q8TCG5	Homo sapiens
MMDBp0192049	Gamma-butyrobetaine dioxygenase	Unknown	O75936	Homo sapiens
MMDBp0192115	Solute carrier organic anion transporter family member 1B1	Transporter	Q9Y6L6	Homo sapiens
MMDBp0192154	Liver carboxylesterase 1	Enzyme	P23141	Homo sapiens
MMDBp0193160	Solute carrier family 22 member 5	Transporter	O76082	Homo sapiens
MMDBp0193543	Solute carrier family 22 member 4	Transporter	Q9H015	Homo sapiens
MMDBp0193677	Mitochondrial carnitine/acylcarnitine carrier protein	Enzyme	O43772	Homo sapiens
MMDBp0193686	Solute carrier family 22 member 16	Transporter	Q86VW1	Homo sapiens
MMDBp0196324	Mitochondrial carnitine/acylcarnitine carrier protein CACL	Unknown	Q8N8R3	Homo sapiens

Human Pathways

Pathways

Name	SMPDB/PathBank
Acylcarnitine 3-Hydroxydecanoylcarnitine
Acylcarnitine (7Z,9Z,12Z,15Z,18Z,21Z)-tetracosa-7,9,12,15,18,21-hexaenoylcarnitine
Acylcarnitine Pentacosanoylcarnitine
Acylcarnitine Hexacosanoylcarnitine
Acylcarnitine (17Z)-Hexacos-17-enoylcarnitine

Metabolic Reactions

Reactions

Reaction ID	Precursor	Product	Enzyme	Reaction Type	Data Source	Reference

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
3-hydroxy-3-methylglutaryl-coa lyase deficiency		Association	Human Blood	HMDB	19893767
3-hydroxy-3-methylglutaryl-coa lyase deficiency	Decreased	Association	Human Blood	HMDB	25557019
Carnitine palmitoyltransferase i deficiency		Association	Human Blood	HMDB	11286380
Diabetes mellitus type 2		Association	Human Urine	HMDB	6810706
L-2-hydroxyglutaric aciduria		Association	Human Urine	HMDB	9187477
L-2-hydroxyglutaric aciduria	Increased	Association	Human Blood	HMDB	9187477
Lung cancer		Association	Human Urine	HMDB	18953024
Mitochondrial trifunctional protein deficiency	Decreased	Association	Human Blood	HMDB	12838198
Obesity		Association	Human Urine	HMDB	26910390
Oculocerebrorenal syndrome	Decreased	Association	Human Blood	HMDB	2017228
Propionic acidemia	Decreased	Association	Human Urine	HMDB	28853722
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)
Obesity	Increased	Association	Human Blood	HMDB	Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children
Obesity	Increased	Association	Human Blood	HMDB	Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Eukaryota/Fungi	Ascomycota	3	Human feces; Human saliva	Unknown
Bacteria	Actinobacteria	20	Human feces; Human ; Human nasopharyngeal swab	Metabolic reconstruction
Bacteria	Firmicutes	194	Human feces; Human ; Human urine; Human mucosa; Human sputum; Human saliva; Human supragingival plaque	Metabolic reconstruction
Bacteria	Proteobacteria	102	Human feces; Human ; Human stool; Human sputum	Metabolic reconstruction
Bacteria	Bacteroidetes	2	Human feces	Metabolic reconstruction

Exposure Sources

Source Type	Source Sub-type	Species	Data Source	Reference

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Std	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Blood	Detected and Quantified			18	22	uM	Infant (0-1 year old)	Male	2,4-dienoyl-CoA reductase deficiency	HMDB	24847004
Human Blood	Detected and Quantified	17				uM	Adult (>18 years old)	Male	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	HMDB	19893767
Human Blood	Detected and Quantified	20.540	1.850			uM	Children (1-13 years old)	Not Specified	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	HMDB	25557019
Human Blood	Detected and Quantified			22000	50000	uM	Not Specified	Not Specified	3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency	HMDB	9727719
Human Blood	Detected and Quantified			43	51	uM	Infant (0-1 year old)	Male	3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)	HMDB	23430856
Human Blood	Detected and Quantified			47	68	uM	Infant (0-1 year old)	Female	3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)	HMDB	11489939
Human Blood	Detected and Quantified	12				uM	Newborn (0-30 days old)	Male	Carnitine palmitoyltransferase deficiency I	HMDB	11286380
Human Blood	Detected and Quantified	15				uM	Newborn (0-30 days old)	Male	Carnitine palmitoyltransferase deficiency I	HMDB	11286380
Human Blood	Detected and Quantified	0				uM	Infant (0-1 year old)	Female	Carnitine transporter defect; primary systemic carnitine deficiency	HMDB	3185635
Human Blood	Detected and Quantified	0.7				uM	Infant (0-1 year old)	Female	Carnitine transporter defect; primary systemic carnitine deficiency	HMDB	3185635
Human Urine	Detected and Quantified	1.517				umol/mmol creatinine	Children (1-13 years old)		Carnitine transporter defect; primary systemic carnitine deficiency	HMDB	7131143
Human Blood	Detected and Quantified	4.2				uM	Children (1-13 years old)	Male	Carnitine transporter defect; primary systemic carnitine deficiency	HMDB	7131143
Human Urine	Detected and Quantified	13.3		11.6	15.1	umol/mmol creatinine	Adult (>18 years old)	Both	Diabetes	HMDB	6810706
Human Urine	Detected and Quantified	41.3		38.3	44.2	umol/mmol creatinine	Adult (>18 years old)	Both	Diabetes	HMDB	6810706
Human Blood	Detected and Quantified	30.2	6.3			uM	Adult (>18 years old)	Female	Early preeclampsia	HMDB	22494326
Human Urine	Detected and Quantified			7	31	umol/mmol creatinine	Children (1-13 years old)	Both	L-2-hydroxyglutaric aciduria	HMDB	9187477
Human Blood	Detected and Quantified			23	50	uM	Children (1-13 years old)	Both	L-2-hydroxyglutaric aciduria	HMDB	9187477
Human Blood	Detected and Quantified	46.8	24.8			uM	Adult (>18 years old)	Female	Late-onset preeclampsia	HMDB	23159745
Human Blood	Detected and Quantified			0	2.2	uM	Infant (0-1 year old)	Female	Long-chain Fatty Acids, Defect in Transport of	HMDB	3185635
Human Urine	Detected and Quantified	7.0		0.0	30.0	umol/mmol creatinine	Not Specified	Both	Lung cancer	HMDB	18953024
Human Blood	Detected and Quantified			23	72	uM	Children (1-13 years old)	Both	Methylmalonic aciduria mitochondrial encephelopathy Leigh-like	HMDB	17301081
Human Blood	Detected and Quantified			10	21	uM	Infant (0-1 year old)		Mitochondrial trifunctional protein deficiency	HMDB	12838198
Human Blood	Detected and Quantified			20.6	33.8	uM	Adult (>18 years old)	Female	Myopathic carnitine deficiency	HMDB	15519880
Human Urine	Detected and Quantified			9.395	121.200	umol/mmol creatinine	Adult (>18 years old)	Female	Myopathic carnitine deficiency	HMDB	15519880
Human Urine	Detected and Quantified	11183.81	6864.25			umol/mmol creatinine	Adolescent (13-18 years old)	Both	Obese	HMDB	26910390
Human Blood	Detected and Quantified			15	34	uM	Children (1-13 years old)	Not Specified	Oculocerebrorenal Syndrome of Lowe	HMDB	2017228
Human Blood	Detected and Quantified			19	31	uM	Adult (>18 years old)	Not Specified	Oculocerebrorenal Syndrome of Lowe	HMDB	2017228
Human Blood	Detected and Quantified			37	48	uM	Infant (0-1 year old)	Not Specified	Oculocerebrorenal Syndrome of Lowe	HMDB	2017228
Human Blood	Detected and Quantified			9	33	uM	Adolescent (13-18 years old)	Not Specified	Oculocerebrorenal Syndrome of Lowe	HMDB	2017228
Human Blood	Detected and Quantified	19.5	8.3			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	23313728
Human Blood	Detected and Quantified	19.98	4.82			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	24704061
Human Blood	Detected and Quantified	24.3	11.7			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	23535240
Human Blood	Detected and Quantified	27.8	20.0			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	23159745
Human Blood	Detected and Quantified	31.8	14.1			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	22494326
Human Urine	Detected and Quantified	7.21				umol/mmol creatinine	Adult (>18 years old)	Not Specified	Propionic acidemia	HMDB	28853722
Human Urine	Detected and Quantified	<100				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	9187477
Human Blood	Detected and Quantified	>25				uM	Infant (0-1 year old)	Not Specified	Normal	HMDB	12838198
Human Urine	Detected and Quantified			0.317	40.928	umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	15519880
Human Urine	Detected and Quantified			0.79	5.66	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	32340350
Human Urine	Detected and Quantified	1.63		0.83	4.31	umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	HMDB	32340350
Human Urine	Detected and Quantified	1.80		0.78	5.85	umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	HMDB	32340350
Human Cerebrospinal fluid (csf)	Detected and Quantified	1.900	0.474			uM	Adult (>18 years old)	Both	Normal	HMDB	22546835
Human Urine	Detected and Quantified	10.733	1.733			umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	HMDB	7131143
Human Blood	Detected and Quantified	14.60	5.43			uM	Children (1-13 years old)	Both	Normal	HMDB	24521011
Human Blood	Detected and Quantified	15.5		13.2	17.7	uM	Newborn (0-30 days old)		Normal	HMDB	25754623
Human Blood	Detected and Quantified	16.28	8.41			uM	Children (1-13 years old)	Both	Normal	HMDB	24521011
Human Feces	Detected and Quantified	19.87	11.39			nmol/g wet feces	Adult (>18 years old)	Both	Normal	HMDB	27624970
Human Blood	Detected and Quantified			25	88	uM	Not Specified	Not Specified	Normal	HMDB	19893767
Human Blood	Detected and Quantified			25.4	54.1	uM	Adult (>18 years old)	Female	Normal	HMDB	15519880
Human Blood	Detected and Quantified			26	66	uM	Infant (0-1 year old)	Not Specified	Normal	HMDB	2017228
Human Blood	Detected and Quantified			26	66	uM	Children (1-13 years old)	Not Specified	Normal	HMDB	2017228
Human Blood	Detected and Quantified			26	70	uM	Children (1-13 years old)	Not Specified	Normal	HMDB	17301081
Human Breast milk	Detected and Quantified	26.2	10.1			uM	Adult (>18 years old)	Female	Normal	HMDB	24027187
Human Blood	Detected and Quantified			28	47	uM	Infant (0-1 year old)	Not Specified	Normal	HMDB	3185635
Human Blood	Detected and Quantified			28.500	109	uM	Infant (0-1 year old)	Not Specified	Normal	HMDB	11489939
Human Blood	Detected and Quantified	29.74	7.55			uM	Adult (>18 years old)	Both	Normal	HMDB	21359215
Human Urine	Detected and Quantified			3.800	5.133	umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	7131143
Human Blood	Detected and Quantified			35	75	uM	Not Specified	Not Specified	Normal	HMDB	9187477
Human Blood	Detected and Quantified	35.3	7.0			uM	Adult (>18 years old)	Both	Normal	HMDB	28278231
Human Blood	Detected and Quantified			37	58	uM	Infant (0-1 year old)	Not Specified	Normal	HMDB	3185635
Human Blood	Detected and Quantified	39.33	13.25			uM	Adult (>18 years old)	Both	Normal	HMDB	26010610
Human Cerebrospinal fluid (csf)	Detected and Quantified	4.0		2.0	9.0	uM	Adult (>18 years old)	Both	Normal	HMDB	1643708
Human Urine	Detected and Quantified	4.5		0.62	15.2	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Blood	Detected and Quantified			40	60	uM	Infant (0-1 year old)	Not Specified	Normal	HMDB	3185635
Human Blood	Detected and Quantified	40.430	3.650			uM	Children (1-13 years old)	Not Specified	Normal	HMDB	25557019
Human Blood	Detected and Quantified	41.2		35.5	47.5	uM	Infant (0-1 year old)		Normal	HMDB	25754623
Human Blood	Detected and Quantified	43.0		26.0	79.0	uM	Adult (>18 years old)	Both	Normal	HMDB	1643708
Human Blood	Detected and Quantified	43.57	9.97			uM	Adult (>18 years old)	Both	Normal	HMDB	26010610
Human Blood	Detected and Quantified	45.7	11.6			uM	Adult (>18 years old)	Both	Normal	HMDB	21359215
Human Urine	Detected and Quantified	5.0		0.7	16.4	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Blood	Detected and Quantified	50	30			uM	Children (1-13 years old)	Not Specified	Normal	HMDB	7131143
Human Urine	Detected and Quantified	6926.20	754.66			umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	HMDB	26910390
Human Feces	Detected and Quantified	8.13	3.11			nmol/g wet feces	Adult (>18 years old)	Both	Normal	HMDB	27624970
Human Urine	Detected and Quantified	17.565	24.221			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 Blood	Detected and Quantified	34.0	8.8			uM	Children (1-13 years old)	Both	Obesity	HMDB	Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children
Human Blood	Detected and Quantified	36.2	8.1			uM	Children (1-13 years old)	Both	Obesity	HMDB	Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children
Human Urine	Detected and Quantified	18.159	16.442			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	18.0	1.2			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	23.5	4.0			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 Blood	Detected and Quantified	33.6	6.2			uM	Children (1-13 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	38.2	5.4			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	5.7	4.8			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	3.17				umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	Shaykhutdinov RA, MacInnis GD, Dowlatabadi R, Weljie AM, Vogel HJ. Quantitative analysis of metabolite concentrations in human urine samples using 13C{1H} NMR spectroscopy. Metabolomics. 2009
Human Saliva	Detected and Quantified	0.656	0.734			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	0.924	0.960			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	5.24	1.035			uM	Adult (>18 years old)	Both	Normal	HMDB	https://doi.org/10.1007/s11306-015-0840-5
Human Blood	Detected and Quantified			19.0	65.0	uM	Adult (>18 years old)	Both	Normal	HMDB	Molecular You (https://molecularyou.com)
Human Adipose tissue	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 Brain	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Erythrocyte	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 Heart	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 Liver	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Lung	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 Semen	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

External Links

HMDB ID

HMDB0000062

DrugBank ID

DB00583

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB000571

KNApSAcK ID

Not Available

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Wikipedia Link

METLIN ID

PubChem Compound

10917

PDB ID

Not Available

ChEBI ID

16347

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

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Shihabi ZK, Oles KS, McCormick CP, Penry JK: Serum and tissue carnitine assay based on dialysis. Clin Chem. 1992 Aug;38(8 Pt 1):1414-7. [PubMed:1643708 ]
Hoppel CL, Genuth SM: Urinary excretion of acetylcarnitine during human diabetic and fasting ketosis. Am J Physiol. 1982 Aug;243(2):E168-72. doi: 10.1152/ajpendo.1982.243.2.E168. [PubMed:6810706 ]
Feinfeld DA, Kurian P, Cheng JT, Dilimetin G, Arriola MR, Ward L, Manis T, Carvounis CP: Effect of oral L-carnitine on serum myoglobin in hemodialysis patients. Ren Fail. 1996 Jan;18(1):91-6. doi: 10.3109/08860229609052778. [PubMed:8820505 ]
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Lenzi A, Sgro P, Salacone P, Paoli D, Gilio B, Lombardo F, Santulli M, Agarwal A, Gandini L: A placebo-controlled double-blind randomized trial of the use of combined l-carnitine and l-acetyl-carnitine treatment in men with asthenozoospermia. Fertil Steril. 2004 Jun;81(6):1578-84. doi: 10.1016/j.fertnstert.2003.10.034. [PubMed:15193480 ]
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Waldner R, Laschan C, Lohninger A, Gessner M, Tuchler H, Huemer M, Spiegel W, Karlic H: Effects of doxorubicin-containing chemotherapy and a combination with L-carnitine on oxidative metabolism in patients with non-Hodgkin lymphoma. J Cancer Res Clin Oncol. 2006 Feb;132(2):121-8. doi: 10.1007/s00432-005-0054-8. Epub 2005 Nov 8. [PubMed:16283381 ]
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Showing metabocard for (R)-Carnitine (MMDBc0033059)

MOL for #<Metabolite:0x00007f46ecc7aff8>

3D MOL for #<Metabolite:0x00007f46ecc7aff8>

3D SDF for #<Metabolite:0x00007f46ecc7aff8>

3D-SDF for #<Metabolite:0x00007f46ecc7aff8>

PDB for #<Metabolite:0x00007f46ecc7aff8>

3D PDB for #<Metabolite:0x00007f46ecc7aff8>

SMILES for #<Metabolite:0x00007f46ecc7aff8>

INCHI for #<Metabolite:0x00007f46ecc7aff8>

Structure for #<Metabolite:0x00007f46ecc7aff8>

3D Structure for #<Metabolite:0x00007f46ecc7aff8>