MiMeDB: Showing metabocard for Galactonic acid (MMDBc0000354)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-12-10 18:35:59 UTC

Update Date

2022-08-31 06:02:31 UTC

Metabolite ID

MMDBc0000354

Metabolite Identification

Common Name

Galactonic acid

Description

Galactonic acid is a sugar acid that is a metabolic breakdown product of galactose. Galactose dehydrogenase is responsible for converting galactose to galactonolactone, which then spontaneously or enzymatically converts to galactonic acid. Once formed, galactonic acid may enter the pentose phosphate pathway. Galactonic acid is increased in red blood cells of galactosemic patients, due to a galactose-1-phosphate uridyltransferase (GALT) deficiency (PMID: 14680973 , OMMBID: The Online Metabolic and Molecular Bases of Inherited Disease, Ch.72). When present in sufficiently high levels, galactonic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of galactonic acid are associated with at least two inborn errors of metabolism, including galactosemia and galactosemia type II. Galactonic acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, liver abnormalities (jaundice), kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated galactosemia. Many affected children with organic acidemias experience intellectual disability or delayed development. High levels of galactonic acid in infants are specifically associated with hepatomegaly (an enlarged liver), cirrhosis, renal failure, cataracts, vomiting, seizure, hypoglycemia, lethargy, brain damage, and ovarian failure.

Structure

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

HEADER    PROTEIN                                 27-MAY-19   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   27-MAY-19         0                                  
HETATM    1  C   UNK     0    8651.3808650.339   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0    8650.0488651.108   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0    8648.7138650.339   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0    8647.3798651.108   0.000  0.00  0.00           C+0
HETATM    5  O   UNK     0    8646.0458650.339   0.000  0.00  0.00           O+0
HETATM    6  O   UNK     0    8648.7138648.799   0.000  0.00  0.00           O+0
HETATM    7  O   UNK     0    8650.0488652.648   0.000  0.00  0.00           O+0
HETATM    8  C   UNK     0    8652.7168651.108   0.000  0.00  0.00           C+0
HETATM    9  C   UNK     0    8654.0518650.339   0.000  0.00  0.00           C+0
HETATM   10  O   UNK     0    8655.3838651.108   0.000  0.00  0.00           O+0
HETATM   11  O   UNK     0    8654.0518648.799   0.000  0.00  0.00           O+0
HETATM   12  O   UNK     0    8652.7168652.648   0.000  0.00  0.00           O+0
HETATM   13  O   UNK     0    8651.3808648.799   0.000  0.00  0.00           O+0
CONECT    1    2    8   13                                                  
CONECT    2    1    3    7                                                  
CONECT    3    2    4    6                                                  
CONECT    4    3    5                                                       
CONECT    5    4                                                            
CONECT    6    3                                                            
CONECT    7    2                                                            
CONECT    8    1    9   12                                                  
CONECT    9    8   10   11                                                  
CONECT   10    9                                                            
CONECT   11    9                                                            
CONECT   12    8                                                            
CONECT   13    1                                                            
MASTER        0    0    0    0    0    0    0    0   13    0   24    0
END

Synonyms

Value	Source
D-Galactonic acid	Kegg
D-Galactonate	Generator
Galactonate	Generator
Galactonic acid, (D)-isomer	HMDB
Galactonic acid, monopotassium salt	HMDB
Galactonic acid, (L)-isomer	HMDB

Molecular Formula

C₆H₁₂O₇

Average Mass

196.1553

Monoisotopic Mass

196.058302738

IUPAC Name

(2R,3S,4S,5R)-2,3,4,5,6-pentahydroxyhexanoic acid

Traditional Name

galactonic acid

CAS Registry Number

Not Available

SMILES

OC[C@@H](O)[C@H](O)[C@H](O)[C@@H](O)C(O)=O

InChI Identifier

InChI=1S/C6H12O7/c7-1-2(8)3(9)4(10)5(11)6(12)13/h2-5,7-11H,1H2,(H,12,13)/t2-,3+,4+,5-/m1/s1

InChI Key

RGHNJXZEOKUKBD-MGCNEYSASA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as medium-chain hydroxy acids and derivatives. These are hydroxy acids with a 6 to 12 carbon atoms long side chain.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Hydroxy acids and derivatives

Sub Class

Medium-chain hydroxy acids and derivatives

Direct Parent

Medium-chain hydroxy acids and derivatives

Alternative Parents

Substituents

Medium-chain hydroxy acid
Medium-chain fatty acid
Beta-hydroxy acid
Hydroxy fatty acid
Alpha-hydroxy acid
Monosaccharide
Fatty acyl
Fatty acid
Secondary alcohol
Polyol
Monocarboxylic acid or derivatives
Carboxylic acid
Carboxylic acid derivative
Organooxygen compound
Hydrocarbon derivative
Organic oxide
Carbonyl group
Primary alcohol
Alcohol
Organic oxygen compound
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

galactonic acid (CHEBI:16534 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Property	Value	Source
Water Solubility	159 g/L	ALOGPS
logP	-2.6	ALOGPS
logP	-3.4	ChemAxon
logS	-0.09	ALOGPS
pKa (Strongest Acidic)	3.39	ChemAxon
pKa (Strongest Basic)	-3	ChemAxon
Physiological Charge	-1	ChemAxon
Hydrogen Acceptor Count	7	ChemAxon
Hydrogen Donor Count	6	ChemAxon
Polar Surface Area	138.45 Å²	ChemAxon
Rotatable Bond Count	5	ChemAxon
Refractivity	38.27 m³·mol⁻¹	ChemAxon
Polarizability	17.07 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	No	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-MS (6 TMS)	splash10-0gb9-1973000000-8a50553fe225b27795e1	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-0gb9-1973000000-8a50553fe225b27795e1	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0002-0931000000-64524701c5b50ec7e875	2017-09-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-06tu-9500000000-abc0b019db16afd3352b	2017-09-01	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (6 TMS) - 70eV, Positive	splash10-03fr-6121297000-698854fa2453487a1d69	2017-10-06	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_6_1) - 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 ("Galactonic acid,6TBDMS,#1" TMS) - 70eV, Positive	Not Available	2021-10-14	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_1_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_4) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_5) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_6) - 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 (TMS_2_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_4) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_5) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_6) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_7) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_8) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_9) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_10) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_11) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, N/A (Annotated)	splash10-056s-0900000000-9dfe4c3a61306fdf2051	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, N/A (Annotated)	splash10-03di-3900000000-a0e411580bfda8c3c12e	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, N/A (Annotated)	splash10-0300-9100000000-8235697c497449a4272d	2012-07-24	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-004j-2900000000-9081b4da0decbd05f2d0	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-03di-9400000000-79c4dcd310d5cacca35c	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-08i9-9000000000-62e401fc518adf3537bd	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-0adu-9600000000-2d2f2523012a7bbe0e8a	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-0a4u-9300000000-00e22f3157480a68e695	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0a4i-9000000000-87d75121bafb2a63a0f8	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-024m-8900000000-7ecf18d54cb462c04558	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-074i-9000000000-a24dd2cb9b57deddeaae	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-08fr-9000000000-ae48df53c8de11d2cae6	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-004i-9100000000-ff1473e44b436e4f8fa5	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-0a6r-9000000000-aac8778b161b37e56a13	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0a6r-9000000000-8920759ff7b62f308018	2021-09-22	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, 5%_DMSO, experimental)		2012-12-04	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	¹H NMR Spectrum (1D, 600 MHz, H₂O, experimental)		2021-10-10	View Spectrum
2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, 5%_DMSO, experimental)		2012-12-05	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm

Biospecimen Locations

Blood
Erythrocyte
Feces
Urine

Tissue Locations

Erythrocyte

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Reaction ID	Precursor	Product	Enzyme	Reaction Type	Data Source	Reference
MMDBr0007821	Galactonic acid	Galactonic acid	Not Available	D-galactonate transport via proton symport, reversible	VMH	30371894
MMDBr0018316	Galactonic acid	2-Keto-3-deoxy-D-gluconic acid	D-galactonate dehydratase	Hydration/dehydration of C-O bond	PathBank	31602464

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
Galactosemia		Association	Human Blood	HMDB	14680973

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Proteobacteria	58	Human ; Human feces; Human feces; pleural fluid plaque; bal; Human stool; Human sputum	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria	Firmicutes	16	Human feces; Human	Metabolic reconstruction
Archaea	Euryarchaeota	1		Metabolic reconstruction
Bacteria	Bacteroidetes	2	Human feces	Metabolic reconstruction
Bacteria	Actinobacteria	1	Human	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 Feces	Detected but not Quantified									HMDB
Human Blood	Detected and Quantified	3.00		0.68	6.47	uM	Children (1-13 years old)	Not Specified	Galactosemia	HMDB	14680973
Human Urine	Detected and Quantified			0	130	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	31506554
Human Blood	Detected and Quantified	2.00		0.69	3.84	uM	Children (1 - 13 years old)	Both	Normal	HMDB	14680973
Human Blood	Detected and Quantified	2.00		0.69	3.84	uM	Children (1-13 years old)	Both	Normal	HMDB	14680973
Human Urine	Detected and Quantified	43.25	28.14			umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	2026685
Human Erythrocyte	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	21389975
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	27543620
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	29808030

External Links

HMDB ID

HMDB0000565

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

3336

PubChem Compound

128869

PDB ID

Not Available

ChEBI ID

16534

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. doi: 10.1016/0378-4347(91)80571-s. [PubMed:2026685 ]
Shinka T, Inoue Y, Peng H, Zhen-Wei X, Ose M, Kuhara T: Urine screening of five-day-old newborns: metabolic profiling of neonatal galactosuria. J Chromatogr B Biomed Sci Appl. 1999 Sep 24;732(2):469-77. doi: 10.1016/s0378-4347(99)00320-5. [PubMed:10517369 ]
Yager CT, Chen J, Reynolds R, Segal S: Galactitol and galactonate in red blood cells of galactosemic patients. Mol Genet Metab. 2003 Nov;80(3):283-9. doi: 10.1016/j.ymgme.2003.08.021. [PubMed:14680973 ]

Showing metabocard for Galactonic acid (MMDBc0000354)

MOL for #<Metabolite:0x00007fe2b478b060>

3D MOL for #<Metabolite:0x00007fe2b478b060>

3D SDF for #<Metabolite:0x00007fe2b478b060>

3D-SDF for #<Metabolite:0x00007fe2b478b060>

PDB for #<Metabolite:0x00007fe2b478b060>

3D PDB for #<Metabolite:0x00007fe2b478b060>

SMILES for #<Metabolite:0x00007fe2b478b060>

INCHI for #<Metabolite:0x00007fe2b478b060>

Structure for #<Metabolite:0x00007fe2b478b060>

3D Structure for #<Metabolite:0x00007fe2b478b060>