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Details on Person UniProt:P05091 ALDH2

Class:IdReferenceGeneProduct:53598
_chainChangeLogtransit peptide:1-17 added on Sat February 7 2015;chain:18-517 added on Sat February 7 2015
_displayNameUniProt:P05091 ALDH2
_timestamp2025-02-21 19:15:17
chaintransit peptide:1-17
chain:18-517
checksumE8F74D44D285A00E
commentFUNCTION Required for clearance of cellular formaldehyde, a cytotoxic and carcinogenic metabolite that induces DNA damage.CATALYTIC ACTIVITY an aldehyde + NAD(+) + H2O = a carboxylate + NADH + 2 H(+)PATHWAY Alcohol metabolism; ethanol degradation; acetate from ethanol: step 2/2.SUBUNIT Homotetramer.SUBCELLULAR LOCATION In response to mitochondrial stress, the precursor protein is ubiquitinated by the SIFI complex in the cytoplasm before mitochondrial import, leading to its degradation (PubMed:38297121). Within the SIFI complex, UBR4 initiates ubiquitin chain that are further elongated or branched by KCMF1 (PubMed:38297121).POLYMORPHISM Genetic variation in ALDH2 is responsible for individual differences in responses to drinking alcohol [MIM:610251]. Allele ALDH2*2 is associated with a very high incidence of acute alcohol intoxication in Orientals and South American Indians, as compared to Caucasians.DISEASE The disease is caused by variants affecting distinct genetic loci, including the gene represented in this entry. AMEDS patients carry ADH5 biallelic variants and homozygous or heterozygous ALDH2 variant p.Glu504Lys, affecting protein activity. Cellular and animal studies demonstrate that the simultaneous loss of ALDH2 and ADH5 activities leads to an increase of cellular formaldehyde sensitivity and multisystem abnormalities including hematopoietic failure.SIMILARITY Belongs to the aldehyde dehydrogenase family.
descriptionrecommendedName: Aldehyde dehydrogenase, mitochondrial ecNumber: 1.2.1.3 alternativeName: ALDH class 2 alternativeName: ALDH-E2 alternativeName: ALDHI
geneNameALDH2
ALDM
identifierP05091
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Alternative splicing
Direct protein sequencing
Disease variant
Dwarfism
Intellectual disability
Mitochondrion
NAD
Oxidoreductase
Proteomics identification
Reference proteome
Transit peptide
Ubl conjugation
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
nameALDH2
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8957714] ENSEMBL:ENSG00000111275 ALDH2 [Homo sapiens]
secondaryIdentifierALDH2_HUMAN
B4DW54
E7EUE5
Q03639
Q6IB13
Q6IV71
sequenceLength517
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:8973161] UniProt:P05091-1 ALDH2 [Homo sapiens]
[ReferenceIsoform:8973162] UniProt:P05091-2 ALDH2 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:71718] ALDH2 [mitochondrial matrix] [Homo sapiens]
[EntityWithAccessionedSequence:8958457] ALDH2 [extracellular region] [Homo sapiens]
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