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Identification
HMDB Protein ID HMDBP00295
Secondary Accession Numbers
  • 5530
Name Aldehyde dehydrogenase, mitochondrial
Synonyms
  1. ALDH class 2
  2. ALDH-E2
  3. ALDHI
Gene Name ALDH2
Protein Type Unknown
Biological Properties
General Function Involved in oxidoreductase activity
Specific Function Not Available
Pathways
  • 2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency
  • 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
  • 3-hydroxyisobutyric acid dehydrogenase deficiency
  • 3-hydroxyisobutyric aciduria
  • 3-Methylcrotonyl Coa Carboxylase Deficiency Type I
  • 3-Methylglutaconic Aciduria Type I
  • 3-Methylglutaconic Aciduria Type III
  • 3-Methylglutaconic Aciduria Type IV
  • 3-Phosphoglycerate dehydrogenase deficiency
  • Arginine and proline metabolism
  • Ascorbate and aldarate metabolism
  • Beta-Alanine Metabolism
  • beta-Alanine metabolism
  • Beta-Ketothiolase Deficiency
  • Carnosinuria, carnosinemia
  • Dihydropyrimidine Dehydrogenase Deficiency (DHPD)
  • Dimethylglycine Dehydrogenase Deficiency
  • Dimethylglycine Dehydrogenase Deficiency
  • Disulfiram Action Pathway
  • Ethanol Degradation
  • ethanol degradation
  • fatty acid metabolism
  • GABA-Transaminase Deficiency
  • Glycerolipid metabolism
  • Glycine and Serine Metabolism
  • Glycolysis / Gluconeogenesis
  • Histidine Metabolism
  • Histidine metabolism
  • Histidinemia
  • Hyperglycinemia, non-ketotic
  • Isobutyryl-coa dehydrogenase deficiency
  • Isovaleric acidemia
  • Isovaleric Aciduria
  • Leigh Syndrome
  • Lysine degradation
  • Maple Syrup Urine Disease
  • Methylmalonate Semialdehyde Dehydrogenase Deficiency
  • Methylmalonic Aciduria
  • Non Ketotic Hyperglycinemia
  • Oxidation of Branched Chain Fatty Acids
  • Pentose and glucuronate interconversions
  • Primary hyperoxaluria II, PH2
  • Propanoate metabolism
  • Propionic Acidemia
  • Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency)
  • Pyruvate Dehydrogenase Complex Deficiency
  • Pyruvate kinase deficiency
  • Pyruvate Metabolism
  • Pyruvate metabolism
  • Sarcosinemia
  • Tryptophan Metabolism
  • Tryptophan metabolism
  • Ureidopropionase Deficiency
  • Valine, Leucine and Isoleucine Degradation
  • Valine, leucine and isoleucine degradation
Reactions
An aldehyde + NAD + Water → a carboxylate + NADH details
2,5-Dioxopentanoate + NADP + Water → Oxoglutaric acid + NADPH + Hydrogen Ion details
Aldehyde + NAD + Water → Fatty acid + NADH + Hydrogen Ion details
Acetaldehyde + NAD + Water → Acetic acid + NADH + Hydrogen Ion details
Acetaldehyde + NADP + Water → Acetic acid + NADPH + Hydrogen Ion details
3-Aminopropionaldehyde + NAD + Water → beta-Alanine + NADH + Hydrogen Ion details
Glyceraldehyde + NAD + Water → Glyceric acid + NADH + Hydrogen Ion details
4-Aminobutyraldehyde + NADP + Water → gamma-Aminobutyric acid + NADPH + Hydrogen Ion details
4-Aminobutyraldehyde + NAD + Water → gamma-Aminobutyric acid + NADH + Hydrogen Ion details
Indoleacetaldehyde + NAD + Water → Indoleacetic acid + NADH + Hydrogen Ion details
2-Propyn-1-al + NAD + Water → Propynoic acid + NADH + Hydrogen Ion details
D-Glucurono-6,3-lactone + NAD + Water → Glucaric acid + NADH + Hydrogen Ion details
4-Trimethylammoniobutanal + NAD + Water → 4-Trimethylammoniobutanoic acid + NADH + Hydrogen Ion details
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH + Hydrogen Ion details
Imidazole-4-acetaldehyde + NAD + Water → Imidazoleacetic acid + NADH + Hydrogen Ion details
3a,7a-Dihydroxy-5b-cholestan-26-al + NAD + Water → 3a,7a-Dihydroxy-5b-cholestanate + NADH + Hydrogen Ion details
5-Hydroxyindoleacetaldehyde + NAD + Water → 5-Hydroxyindoleacetic acid + Hydrogen Ion + NADH details
N4-Acetylaminobutanal + NAD + Water → 4-Acetamidobutanoic acid + NADH + Hydrogen Ion details
trans-3-Chloroallyl aldehyde + Water → trans-3-Chloroacrylic acid + Hydrogen Ion details
cis-3-Chloroallyl aldehyde + Water → cis-3-Chloroacrylic acid + Hydrogen Ion details
Chloroacetaldehyde + NAD + Water → Chloroacetic acid + NADH + Hydrogen Ion details
Perillyl aldehyde + Water + NAD → Perillic acid + NADH + Hydrogen Ion details
GO Classification
Biological Process
ethanol catabolic process
neurotransmitter biosynthetic process
xenobiotic metabolic process
ethanol oxidation
carbohydrate metabolic process
Cellular Component
mitochondrial matrix
Function
catalytic activity
oxidoreductase activity
Molecular Function
electron carrier activity
aldehyde dehydrogenase (NAD) activity
aldehyde dehydrogenase [NAD(P)+] activity
Process
metabolic process
oxidation reduction
Cellular Location
  1. Mitochondrion matrix
Gene Properties
Chromosome Location 12
Locus 12q24.2
SNPs ALDH2
Gene Sequence
>1551 bp
ATGTTGCGCGCTGCCGCCGCTCGGGCCCCGCCTGGCCGCCGCCTCTTGTCAGCCGCCGCC
ACCCAGGCCGTGCCTGCCCCCAACCAGCAGCCCGAGGTCTTCTGCAACCAGATTTTCATA
AACAATGAATGGCACGATGCCGTCAGCAGGAAAACATTCCCCACCGTCAATCCGTCCACT
GGAGAGGTCATCTGTCAGGTAGCTGAAGGGGACAAGGAAGATGTGGACAAGGCACGTGAA
GGCCGCCCGGGCGCCTTCCAGCTGGGCTCACCTTGGCGCCGCATGGACGCATCACACAGC
GGCCGGCTGCTGAACCGCCTGGCCGATCTGATCGAGCGGGACCGGACCTACCTGGCGGCC
TTGGAGACCCTGGACAATGGCAAGCCCTATGTCATCTCCTACCTGGTGGATTTGGACATG
GTCCTCAAATGTCTCCGGTATTATGCCGGCTGGGCTGATAAGTACCACGGGAAAACCATC
CCCATTGACGGAGACTTCTTCAGCTACACACGCCATGAACCTGTGGGGGTGTGCGGGCAG
ATCATTCCGTGGAATTTCCCGCTCCTGATGCAAGCATGGAAGCTGGGCCCAGCCTTGGCA
ACTGGAAACGTGGTTGTGATGAAGGTAGCTGAGCAGACACCCCTCACCGCCCTCTATGTG
GCCAACCTGATCAAGGAGGCTGGCTTTCCCCCTGGTGTGGTCAACATTGTGCCTGGATTT
GGCCCCACGGCTGGGGCCGCCATTGCCTCCCATGAGGATGTGGACAAAGTGGCATTCACA
GGCTCCACTGAGATTGGCCGCGTAATCCAGGTTGCTGCTGGGAGCAGCAACCTCAAGAGA
GTGACCTTGGAGCTGGGGGGGAAGAGCCCCAACATCATCATGTCAGATGCCGATATGGAT
TGGGCCGTGGAACAGGCCCACTTCGCCCTGTTCTTCAACCAGGGCCAGTGCTGCTGTGCC
GGCTCCCGGACCTTCGTGCAGGAGGACATCTATGATGAGTTTGTGGTGCGGAGCGTTGCC
CGGGCCAAGTCTCGGGTGGTCGGGAACCCCTTTGATAGCAAGACCGAGCAGGGGCCGCAG
GTGGATGAAACTCAGTTTAAGAAGATCCTCGGCTACATCAACACGGGGAAGCAAGAGGGG
GCGAAGCTGCTGTGTGGTGGGGGCATTGCTGCTGACCGTGGTTACTTCATCCAGCCCACT
GTGTTTGGAGATGTGCAGGATGGCATGACCATCGCCAAGGAGGAGATCTTCGGGCCAGTG
ATGCAGATCCTGAAGTTCAAGACCATAGAGGAGGTTGTTGGGAGAGCCAACAATTCCACG
TACGGGCTGGCCGCAGCTGTCTTCACAAAGGATTTGGACAAGGCCAATTACCTGTCCCAG
GCCCTCCAGGCGGGCACTGTGTGGGTCAACTGCTATGATGTGTTTGGAGCCCAGTCACCC
TTTGGTGGCTACAAGATGTCGGGGAGTGGCCGGGAGTTGGGCGAGTACGGGCTGCAGGCA
TACACTGAAGTGAAAACTGTCACAGTCAAAGTGCCTCAGAAGAACTCATAA
Protein Properties
Number of Residues 517
Molecular Weight 56380.93
Theoretical pI 7.045
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Aldehyde dehydrogenase, mitochondrial
MLRAAARFGPRLGRRLLSAAATQAVPAPNQQPEVFCNQIFINNEWHDAVSRKTFPTVNPS
TGEVICQVAEGDKEDVDKAVKAARAAFQLGSPWRRMDASHRGRLLNRLADLIERDRTYLA
ALETLDNGKPYVISYLVDLDMVLKCLRYYAGWADKYHGKTIPIDGDFFSYTRHEPVGVCG
QIIPWNFPLLMQAWKLGPALATGNVVVMKVAEQTPLTALYVANLIKEAGFPPGVVNIVPG
FGPTAGAAIASHEDVDKVAFTGSTEIGRVIQVAAGSSNLKRVTLELGGKSPNIIMSDADM
DWAVEQAHFALFFNQGQCCCAGSRTFVQEDIYDEFVERSVARAKSRVVGNPFDSKTEQGP
QVDETQFKKILGYINTGKQEGAKLLCGGGIAADRGYFIQPTVFGDVQDGMTIAKEEIFGP
VMQILKFKTIEEVVGRANNSTYGLAAAVFTKDLDKANYLSQALQAGTVWVNCYDVFGAQS
PFGGYKMSGSGRELGEYGLQAYTEVKTVTVKVPQKNS
GenBank ID Protein 28606
UniProtKB/Swiss-Prot ID P05091
UniProtKB/Swiss-Prot Entry Name ALDH2_HUMAN
PDB IDs
GenBank Gene ID X05409
GeneCard ID ALDH2
GenAtlas ID ALDH2
HGNC ID HGNC:404
References
General References
  1. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  2. Aboulaich N, Vainonen JP, Stralfors P, Vener AV: Vectorial proteomics reveal targeting, phosphorylation and specific fragmentation of polymerase I and transcript release factor (PTRF) at the surface of caveolae in human adipocytes. Biochem J. 2004 Oct 15;383(Pt 2):237-48. [PubMed:15242332 ]
  3. Yoshida A, Ikawa M, Hsu LC, Tani K: Molecular abnormality and cDNA cloning of human aldehyde dehydrogenases. Alcohol. 1985 Jan-Feb;2(1):103-6. [PubMed:4015823 ]
  4. Hsu LC, Tani K, Fujiyoshi T, Kurachi K, Yoshida A: Cloning of cDNAs for human aldehyde dehydrogenases 1 and 2. Proc Natl Acad Sci U S A. 1985 Jun;82(11):3771-5. [PubMed:2987944 ]
  5. Braun T, Bober E, Singh S, Agarwal DP, Goedde HW: Evidence for a signal peptide at the amino-terminal end of human mitochondrial aldehyde dehydrogenase. FEBS Lett. 1987 May 11;215(2):233-6. [PubMed:3582651 ]
  6. Braun T, Bober E, Singh S, Agarwal DP, Goedde HW: Isolation and sequence analysis of a full length cDNA clone coding for human mitochondrial aldehyde dehydrogenase. Nucleic Acids Res. 1987 Apr 10;15(7):3179. [PubMed:3562250 ]
  7. Hsu LC, Bendel RE, Yoshida A: Genomic structure of the human mitochondrial aldehyde dehydrogenase gene. Genomics. 1988 Jan;2(1):57-65. [PubMed:2838413 ]
  8. Hempel J, Kaiser R, Jornvall H: Mitochondrial aldehyde dehydrogenase from human liver. Primary structure, differences in relation to the cytosolic enzyme, and functional correlations. Eur J Biochem. 1985 Nov 15;153(1):13-28. [PubMed:4065146 ]
  9. Agarwal DP, Goedde HW: Human aldehyde dehydrogenase isozymes and alcohol sensitivity. Isozymes Curr Top Biol Med Res. 1987;16:21-48. [PubMed:3610592 ]
  10. Hempel J, Hoog JO, Jornvall H: Mitochondrial aldehyde dehydrogenase. Homology of putative targeting sequence to that of carbamyl phosphate synthetase I revealed by correlation of cDNA and protein data. FEBS Lett. 1987 Sep 28;222(1):95-8. [PubMed:3653404 ]
  11. Ni L, Zhou J, Hurley TD, Weiner H: Human liver mitochondrial aldehyde dehydrogenase: three-dimensional structure and the restoration of solubility and activity of chimeric forms. Protein Sci. 1999 Dec;8(12):2784-90. [PubMed:10631996 ]
  12. Yoshida A, Huang IY, Ikawa M: Molecular abnormality of an inactive aldehyde dehydrogenase variant commonly found in Orientals. Proc Natl Acad Sci U S A. 1984 Jan;81(1):258-61. [PubMed:6582480 ]
  13. Novoradovsky A, Tsai SJ, Goldfarb L, Peterson R, Long JC, Goldman D: Mitochondrial aldehyde dehydrogenase polymorphism in Asian and American Indian populations: detection of new ALDH2 alleles. Alcohol Clin Exp Res. 1995 Oct;19(5):1105-10. [PubMed:8561277 ]