ACADS
entrez
35
canonical
uniprot
P16219
canonical
ensembl
ENSG00000122971
resolver
ensp
ENSP00000242592
resolver
ensp
ENSP00000401045
resolver
enst
ENST00000242592
resolver
enst
ENST00000411593
resolver
genesymbol
ACADS
resolver
hgnc
HGNC:90
resolver
uniprot
B4DUH1
resolver
uniprot
E5KSD5
resolver
uniprot
E9PE82
resolver
uniprot
P78331
resolver
uniprot_entry
ACADS_HUMAN
resolver
uniprot_entry
B4DUH1_HUMAN
resolver
uniprot_entry
E5KSD5_HUMAN
resolver
uniprot_entry
E9PE82_HUMAN
resolver
uniprot_sec
P78331
resolver
amino_acid_sequence
MAAALLARASGPARRALCPRAWRQLHTIYQSVELPETHQMLLQTCRDFAEKELFPIAAQVDKEHLFPAAQVKKMGGLGLLAMDVPEELGGAGLDYLAYAIAMEEISRGCASTGVIMSVNNSLYLGPILKFGSKEQKQAWVTPFTSGDKIGCFALSEPGNGSDAGAASTTARAEGDSWVLNGTKAWITNAWEASAAVVFASTDRALQNKGISAFLVPMPTPGLTLGKKEDKLGIRGSSTANLIFEDCRIPKDSILGEPGMGFKIAMQTLDMGRIGIASQALGIAQTALDCAVNYAENRMAFGAPLTKLQVIQFKLADMALALESARLLTWRAAMLKDNKKPFIKEAAMAKLAASEAATAISHQAIQILGGMGYVTEMPAERHYRDARITEIYEGTSEIQRLVIAGHLLRSYRS
uniprot
proteins
disease_involvement
DISEASE: Acyl-CoA dehydrogenase short-chain deficiency (ACADSD) [MIM:201470]: An inborn error of mitochondrial fatty acid beta-oxidation resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in adults. {ECO:0000269|PubMed:11134486, ECO:0000269|PubMed:1692038, ECO:0000269|PubMed:9499414}. Note=The disease is caused by variants affecting the gene represented in this entry.
uniprot
proteins
ec_number
1.3.8.1
uniprot
proteins
function
FUNCTION: Short-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats (By similarity). The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA (By similarity). Among the different mitochondrial acyl-CoA dehydrogenases, short-chain specific acyl-CoA dehydrogenase acts specifically on acyl-CoAs with saturated 4 to 6 carbons long primary chains (PubMed:11134486, PubMed:21237683). {ECO:0000250|UniProtKB:P15651, ECO:0000269|PubMed:11134486, ECO:0000269|PubMed:21237683}.
uniprot
proteins
mass_dalton
44297
uniprot
proteins
pathway_participation
PATHWAY: Lipid metabolism; mitochondrial fatty acid beta-oxidation. {ECO:0000305|PubMed:11134486}.
uniprot
proteins
protein_family
Acyl-CoA dehydrogenase family
uniprot
proteins
pubmed
11134486
uniprot
proteins
pubmed
1286669
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
1692038
uniprot
proteins
pubmed
21237683
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
2565344
uniprot
proteins
pubmed
25944712
uniprot
proteins
pubmed
9383286
uniprot
proteins
pubmed
9499414
uniprot
proteins
sequence_length
412
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion matrix {ECO:0000250|UniProtKB:Q3ZBF6}.
uniprot
proteins