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Showing metabocard for Methylmalonic acid (HMDB0000202)

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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-05-30 20:55:53 UTC
HMDB IDHMDB0000202
Secondary Accession Numbers
  • HMDB00202
Metabolite Identification
Common NameMethylmalonic acid
Description
Structure
Thumb
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000202 (Methylmalonic acid)

  Mrv1652305271900212D          

  8  7  0  0  0  0            999 V2000
    2.0625    0.7145    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2375    0.7145    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.8250    1.4289    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2375    2.1434    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.0000    1.4289    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.8250    0.0000    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2375   -0.7145    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.0000    0.0000    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  2  0  0  0  0
  3  5  1  0  0  0  0
  2  6  1  0  0  0  0
  6  7  2  0  0  0  0
  6  8  1  0  0  0  0
M  END
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3D MOL for HMDB0000202 (Methylmalonic acid)

HMDB0000202
     RDKit          3D
Methylmalonic acid
 14 13  0  0  0  0  0  0  0  0999 V2000
   -0.0713   -0.4912    1.2791 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1048   -0.3766   -0.2320 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9010    0.8030   -0.5919 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3350    1.7138   -1.2495 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2207    0.9632   -0.2511 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.2455   -0.3645   -0.8307 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.5790   -1.2719   -1.6650 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.1941    0.5808   -0.5449 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1102    0.4838    1.7864 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9342   -1.1118    1.6506 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.8573   -1.0216    1.5594 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6628   -1.2733   -0.5937 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.9927    0.4363   -0.6617 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.4569    0.9300    0.3448 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  2  0
  3  5  1  0
  2  6  1  0
  6  7  2  0
  6  8  1  0
  1  9  1  0
  1 10  1  0
  1 11  1  0
  2 12  1  0
  5 13  1  0
  8 14  1  0
M  END
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3D SDF for HMDB0000202 (Methylmalonic acid)

  Mrv1652305271900212D          

  8  7  0  0  0  0            999 V2000
    2.0625    0.7145    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2375    0.7145    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.8250    1.4289    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2375    2.1434    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.0000    1.4289    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.8250    0.0000    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2375   -0.7145    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.0000    0.0000    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  2  0  0  0  0
  3  5  1  0  0  0  0
  2  6  1  0  0  0  0
  6  7  2  0  0  0  0
  6  8  1  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000202

> <DATABASE_NAME>
hmdb

> <SMILES>
CC(C(O)=O)C(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8)

> <INCHI_KEY>
ZIYVHBGGAOATLY-UHFFFAOYSA-N

> <FORMULA>
C4H6O4

> <MOLECULAR_WEIGHT>
118.088

> <EXACT_MASS>
118.02660868

> <JCHEM_ACCEPTOR_COUNT>
4

> <JCHEM_ATOM_COUNT>
14

> <JCHEM_AVERAGE_POLARIZABILITY>
10.057853065917067

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
2-methylpropanedioic acid

> <ALOGPS_LOGP>
0.17

> <JCHEM_LOGP>
0.21186901000000008

> <ALOGPS_LOGS>
0.10

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-2

> <JCHEM_PKA>
6.256772105384455

> <JCHEM_PKA_STRONGEST_ACIDIC>
2.4752703991264853

> <JCHEM_POLAR_SURFACE_AREA>
74.6

> <JCHEM_REFRACTIVITY>
23.5623

> <JCHEM_ROTATABLE_BOND_COUNT>
2

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.49e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
methylmalonic acid

> <JCHEM_VEBER_RULE>
0

$$$$
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3D-SDF for HMDB0000202 (Methylmalonic acid)

HMDB0000202
     RDKit          3D
Methylmalonic acid
 14 13  0  0  0  0  0  0  0  0999 V2000
   -0.0713   -0.4912    1.2791 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1048   -0.3766   -0.2320 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9010    0.8030   -0.5919 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3350    1.7138   -1.2495 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2207    0.9632   -0.2511 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.2455   -0.3645   -0.8307 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.5790   -1.2719   -1.6650 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.1941    0.5808   -0.5449 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1102    0.4838    1.7864 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9342   -1.1118    1.6506 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.8573   -1.0216    1.5594 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6628   -1.2733   -0.5937 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.9927    0.4363   -0.6617 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.4569    0.9300    0.3448 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  2  0
  3  5  1  0
  2  6  1  0
  6  7  2  0
  6  8  1  0
  1  9  1  0
  1 10  1  0
  1 11  1  0
  2 12  1  0
  5 13  1  0
  8 14  1  0
M  END
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PDB for HMDB0000202 (Methylmalonic acid)

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       3.850   1.334   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0       2.310   1.334   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0       1.540   2.667   0.000  0.00  0.00           C+0
HETATM    4  O   UNK     0       2.310   4.001   0.000  0.00  0.00           O+0
HETATM    5  O   UNK     0       0.000   2.667   0.000  0.00  0.00           O+0
HETATM    6  C   UNK     0       1.540   0.000   0.000  0.00  0.00           C+0
HETATM    7  O   UNK     0       2.310  -1.334   0.000  0.00  0.00           O+0
HETATM    8  O   UNK     0       0.000   0.000   0.000  0.00  0.00           O+0
CONECT    1    2                                                            
CONECT    2    1    3    6                                                  
CONECT    3    2    4    5                                                  
CONECT    4    3                                                            
CONECT    5    3                                                            
CONECT    6    2    7    8                                                  
CONECT    7    6                                                            
CONECT    8    6                                                            
MASTER        0    0    0    0    0    0    0    0    8    0   14    0
END
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3D PDB for HMDB0000202 (Methylmalonic acid)

COMPND    HMDB0000202
HETATM    1  C1  UNL     1      -0.071  -0.491   1.279  1.00  0.00           C  
HETATM    2  C2  UNL     1      -0.105  -0.377  -0.232  1.00  0.00           C  
HETATM    3  C3  UNL     1      -0.901   0.803  -0.592  1.00  0.00           C  
HETATM    4  O1  UNL     1      -0.335   1.714  -1.249  1.00  0.00           O  
HETATM    5  O2  UNL     1      -2.221   0.963  -0.251  1.00  0.00           O  
HETATM    6  C4  UNL     1       1.246  -0.364  -0.831  1.00  0.00           C  
HETATM    7  O3  UNL     1       1.579  -1.272  -1.665  1.00  0.00           O  
HETATM    8  O4  UNL     1       2.194   0.581  -0.545  1.00  0.00           O  
HETATM    9  H1  UNL     1      -0.110   0.484   1.786  1.00  0.00           H  
HETATM   10  H2  UNL     1      -0.934  -1.112   1.651  1.00  0.00           H  
HETATM   11  H3  UNL     1       0.857  -1.022   1.559  1.00  0.00           H  
HETATM   12  H4  UNL     1      -0.663  -1.273  -0.594  1.00  0.00           H  
HETATM   13  H5  UNL     1      -2.993   0.436  -0.662  1.00  0.00           H  
HETATM   14  H6  UNL     1       2.457   0.930   0.345  1.00  0.00           H  
CONECT    1    2    9   10   11
CONECT    2    3    6   12
CONECT    3    4    4    5
CONECT    5   13
CONECT    6    7    7    8
CONECT    8   14
END
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SMILES for HMDB0000202 (Methylmalonic acid)

CC(C(O)=O)C(O)=O
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INCHI for HMDB0000202 (Methylmalonic acid)

InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8)
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View in JSmolView Stereo Labels
Synonyms
Chemical FormulaC4H6O4
Average Molecular Weight118.088
Monoisotopic Molecular Weight118.02660868
IUPAC Name2-methylpropanedioic acid
Traditional Namemethylmalonic acid
CAS Registry Number516-05-2
SMILES
CC(C(O)=O)C(O)=O
InChI Identifier
InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8)
InChI KeyZIYVHBGGAOATLY-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassDicarboxylic acids and derivatives
Direct ParentDicarboxylic acids and derivatives
Alternative Parents
Substituents
  • 1,3-dicarbonyl compound
  • Dicarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Health effect
Disposition
Biological locationSource
Process
Naturally occurring process
Role
Industrial applicationIndirect biological roleBiological role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point135 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility679 mg/mLNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Locations
  • Kidney
  • Liver
  • Placenta
Pathways
Normal Concentrations
Abnormal Concentrations
Associated Disorders and Diseases
Disease References
Vitamin B12 deficiency
  1. Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [PubMed:8412012 ]
  2. Stabler SP, Marcell PD, Podell ER, Allen RH, Lindenbaum J: Assay of methylmalonic acid in the serum of patients with cobalamin deficiency using capillary gas chromatography-mass spectrometry. J Clin Invest. 1986 May;77(5):1606-12. [PubMed:3700655 ]
Malonyl-Coa decarboxylase deficiency
  1. Polinati PP, Valanne L, Tyni T: Malonyl-CoA decarboxylase deficiency: long-term follow-up of a patient new clinical features and novel mutations. Brain Dev. 2015 Jan;37(1):107-13. doi: 10.1016/j.braindev.2014.02.001. Epub 2014 Mar 7. [PubMed:24613099 ]
  2. Haan EA, Scholem RD, Croll HB, Brown GK: Malonyl coenzyme A decarboxylase deficiency. Clinical and biochemical findings in a second child with a more severe enzyme defect. Eur J Pediatr. 1986 Apr;144(6):567-70. [PubMed:3709568 ]
Methylmalonic aciduria mitochondrial encephelopathy Leigh-like
  1. Carrozzo R, Dionisi-Vici C, Steuerwald U, Lucioli S, Deodato F, Di Giandomenico S, Bertini E, Franke B, Kluijtmans LA, Meschini MC, Rizzo C, Piemonte F, Rodenburg R, Santer R, Santorelli FM, van Rooij A, Vermunt-de Koning D, Morava E, Wevers RA: SUCLA2 mutations are associated with mild methylmalonic aciduria, Leigh-like encephalomyopathy, dystonia and deafness. Brain. 2007 Mar;130(Pt 3):862-74. Epub 2007 Feb 14. [PubMed:17301081 ]
Methylmalonic aciduria, cblA type
  1. Keyfi F, Talebi S, Varasteh AR: Methylmalonic Acidemia Diagnosis by Laboratory Methods. Rep Biochem Mol Biol. 2016 Oct;5(1):1-14. [PubMed:28070528 ]
Methylmalonic aciduria, cblB type
  1. Keyfi F, Talebi S, Varasteh AR: Methylmalonic Acidemia Diagnosis by Laboratory Methods. Rep Biochem Mol Biol. 2016 Oct;5(1):1-14. [PubMed:28070528 ]
Mitochondrial encephalomyopaththy with elevanted methylmalonic acid, SUCLA2
  1. Ostergaard E, Hansen FJ, Sorensen N, Duno M, Vissing J, Larsen PL, Faeroe O, Thorgrimsson S, Wibrand F, Christensen E, Schwartz M: Mitochondrial encephalomyopathy with elevated methylmalonic acid is caused by SUCLA2 mutations. Brain. 2007 Mar;130(Pt 3):853-61. Epub 2007 Feb 7. [PubMed:17287286 ]
Alzheimer's disease
  1. Serot JM, Barbe F, Arning E, Bottiglieri T, Franck P, Montagne P, Nicolas JP: Homocysteine and methylmalonic acid concentrations in cerebrospinal fluid: relation with age and Alzheimer's disease. J Neurol Neurosurg Psychiatry. 2005 Nov;76(11):1585-7. [PubMed:16227558 ]
  2. Kristensen MO, Gulmann NC, Christensen JE, Ostergaard K, Rasmussen K: Serum cobalamin and methylmalonic acid in Alzheimer dementia. Acta Neurol Scand. 1993 Jun;87(6):475-81. [PubMed:8356878 ]
Cobalamin malabsorption
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Propionic acidemia
  1. Riemersma M, Hazebroek MR, Helderman-van den Enden ATJM, Salomons GS, Ferdinandusse S, Brouwers MCGJ, van der Ploeg L, Heymans S, Glatz JFC, van den Wijngaard A, Krapels IPC, Bierau J, Brunner HG: Propionic acidemia as a cause of adult-onset dilated cardiomyopathy. Eur J Hum Genet. 2017 Nov;25(11):1195-1201. doi: 10.1038/ejhg.2017.127. Epub 2017 Aug 30. [PubMed:28853722 ]
Fumarase deficiency
  1. Bastug O, Kardas F, Ozturk MA, Halis H, Memur S, Korkmaz L, Tag Z, Gunes T: A rare cause of opistotonus; fumaric aciduria: The first case presentation in Turkey. Turk Pediatri Ars. 2014 Mar 1;49(1):74-6. doi: 10.5152/tpa.2014.442. eCollection 2014 Mar. [PubMed:26078636 ]
Transcobalamin II deficiency
  1. Bibi H, Gelman-Kohan Z, Baumgartner ER, Rosenblatt DS: Transcobalamin II deficiency with methylmalonic aciduria in three sisters. J Inherit Metab Dis. 1999 Oct;22(7):765-72. [PubMed:10518276 ]
Cobalamin F disease (cblF)
  1. Alfadhel M, Lillquist YP, Davis C, Junker AK, Stockler-Ipsiroglu S: Eighteen-year follow-up of a patient with cobalamin F disease (cblF): report and review. Am J Med Genet A. 2011 Oct;155A(10):2571-7. doi: 10.1002/ajmg.a.34220. Epub 2011 Sep 9. [PubMed:21910240 ]
Combined malonic and methylmalonic aciduria
  1. Sloan JL, Johnston JJ, Manoli I, Chandler RJ, Krause C, Carrillo-Carrasco N, Chandrasekaran SD, Sysol JR, O'Brien K, Hauser NS, Sapp JC, Dorward HM, Huizing M, Barshop BA, Berry SA, James PM, Champaigne NL, de Lonlay P, Valayannopoulos V, Geschwind MD, Gavrilov DK, Nyhan WL, Biesecker LG, Venditti CP: Exome sequencing identifies ACSF3 as a cause of combined malonic and methylmalonic aciduria. Nat Genet. 2011 Aug 14;43(9):883-6. doi: 10.1038/ng.908. [PubMed:21841779 ]
Associated OMIM IDs
  • 248360 (Malonyl-Coa decarboxylase deficiency)
  • 251100 (Methylmalonic aciduria, cblA type)
  • 251110 (Methylmalonic aciduria, cblB type)
  • 612073 (Mitochondrial encephalomyopaththy with elevanted methylmalonic acid, SUCLA2)
  • 104300 (Alzheimer's disease)
  • 610247 (Eosinophilic esophagitis)
  • 606054 (Propionic acidemia)
  • 606812 (Fumarase deficiency)
  • 275350 (Transcobalamin II deficiency)
  • 277380 (Cobalamin F disease (cblF))
  • 614265 (Combined malonic and methylmalonic aciduria)
DrugBank IDDB04183
Phenol Explorer Compound IDNot Available
FooDB IDFDB021905
KNApSAcK IDNot Available
Chemspider ID473
KEGG Compound IDC02170
BioCyc IDCPD-546
BiGG IDNot Available
Wikipedia LinkMethylmalonic_acid
METLIN ID3712
PubChem Compound487
PDB IDNot Available
ChEBI ID30860
Food Biomarker OntologyNot Available
VMH IDHC00900
MarkerDB IDMDB00000097
Good Scents IDNot Available
References
Synthesis ReferenceEdamura, Koji; Arai, Takeshi. Malonic and methylmalonic acids from b-hydroxypropionic acids. Jpn. Kokai Tokkyo Koho (1979), 5 pp.
Material Safety Data Sheet (MSDS)Not Available
General References

Enzymes

Enzyme Details
1. Aldehyde oxidase
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
AOX1
Uniprot ID:
Q06278
Molecular weight:
147916.735
Reactions
Methylmalonic acid + Hydrogen peroxide → (S)-Methylmalonic acid semialdehyde + Oxygen + Waterdetails
Enzyme Details
2. 4-trimethylaminobutyraldehyde dehydrogenase
General function:
Involved in oxidoreductase activity
Specific function:
Converts gamma-trimethylaminobutyraldehyde into gamma-butyrobetaine. Catalyzes the irreversible oxidation of a broad range of aldehydes to the corresponding acids in an NAD-dependent reaction.
Gene Name:
ALDH9A1
Uniprot ID:
P49189
Molecular weight:
56291.485
Reactions
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH + Hydrogen Iondetails
Enzyme Details
3. Alpha-aminoadipic semialdehyde dehydrogenase
General function:
Involved in oxidoreductase activity
Specific function:
Multifunctional enzyme mediating important protective effects. Metabolizes betaine aldehyde to betaine, an important cellular osmolyte and methyl donor. Protects cells from oxidative stress by metabolizing a number of lipid peroxidation-derived aldehydes. Involved in lysine catabolism.
Gene Name:
ALDH7A1
Uniprot ID:
P49419
Molecular weight:
58486.74
Reactions
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH + Hydrogen Iondetails
Enzyme Details
4. Aldehyde dehydrogenase family 1 member A3
General function:
Involved in oxidoreductase activity
Specific function:
Recognizes as substrates free retinal and cellular retinol-binding protein-bound retinal. Seems to be the key enzyme in the formation of an RA gradient along the dorso-ventral axis during the early eye development and also in the development of the olfactory system (By similarity).
Gene Name:
ALDH1A3
Uniprot ID:
P47895
Molecular weight:
56107.995
Enzyme Details
5. Aldehyde dehydrogenase, mitochondrial
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
ALDH2
Uniprot ID:
P05091
Molecular weight:
56380.93
Reactions
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH + Hydrogen Iondetails
Enzyme Details
6. Fatty aldehyde dehydrogenase
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the oxidation of long-chain aliphatic aldehydes to fatty acids. Active on a variety of saturated and unsaturated aliphatic aldehydes between 6 and 24 carbons in length. Responsible for conversion of the sphingosine 1-phosphate (S1P) degradation product hexadecenal to hexadecenoic acid.
Gene Name:
ALDH3A2
Uniprot ID:
P51648
Molecular weight:
54847.36
Reactions
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH + Hydrogen Iondetails
Enzyme Details
7. Aldehyde dehydrogenase X, mitochondrial
General function:
Involved in oxidoreductase activity
Specific function:
ALDHs play a major role in the detoxification of alcohol-derived acetaldehyde. They are involved in the metabolism of corticosteroids, biogenic amines, neurotransmitters, and lipid peroxidation.
Gene Name:
ALDH1B1
Uniprot ID:
P30837
Molecular weight:
57248.96
Reactions
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH + Hydrogen Iondetails