Hadha
entrez
170670
canonical
uniprot
Q64428
canonical
ensembl
ENSRNOG00000024629
resolver
ensembl
ENSRNOG00055020140
resolver
ensembl
ENSRNOG00060011914
resolver
ensembl
ENSRNOG00065024062
resolver
ensp
ENSRNOP00000107572
resolver
ensp
ENSRNOP00055027879
resolver
ensp
ENSRNOP00060015851
resolver
ensp
ENSRNOP00065033762
resolver
enst
ENSRNOT00000142694
resolver
enst
ENSRNOT00055034408
resolver
enst
ENSRNOT00060020179
resolver
enst
ENSRNOT00065041333
resolver
genesymbol
Hadha
resolver
uniprot
Q5BIZ5
resolver
uniprot_entry
ECHA_RAT
resolver
uniprot_sec
Q5BIZ5
resolver
amino_acid_sequence
MVASRAIGSLSRFSAFRILRSRGCICHSFTTSSALLSRTHINYGVKGDVAVIRINSPNSKVNTLNKEVQSEFVEVMNEIWANDQIRSAVLISSKPGCFVAGADINMLASCTTPQEAARISQEGQKMFEKLEKSPKPVVAAISGSCLGGGLELAIACQYRIATKDRKTVLGVPEVLLGILPGAGGTQRLPKMVGVPAAFDMMLTGRNIRADRAKKMGLVDQLVDPLGPGIKSPEERTIEYLEEVAVNFAKGLADRKVSAKQSKGLMEKLTSYAMTIPFVRQQVYKTVEEKVKKQTKGLYPAPLKIIDAVKTGLEQGNDAGYLAESEKFGELALTKESKALMGLYNGQVLCKKNKFGAPQKTVQQLAILGAGLMGAGIAQVSVDKGLKTLLKDTTVTGLGRGQQQVFKGLNDKVKKKALTSFERDSIFSNLIGQLDYKGFEKADMVIEAVFEDLAVKHKVLKEVESVTPEHCIFASNTSALPINQIAAVSQRPEKVIGMHYFSPVDKMQLLEIITTDKTSKDTTASAVAVGLKQGKVIIVVKDGPGFYTTRCLAPMMSEVIRILQEGVDPKKLDALTTGFGFPVGAATLADEVGIDVAQHVAEDLGKAFGERFGGGSVELLKLMVSKGFLGRKSGKGFYIYQSGSKNKNLNSEIDNILVNLRLPAKPEVSSDEDIQYRVITRFVNEAVLCLQEGILATPEEGDIGAVFGLGFPPCLGGPFRFVDLYGAQKVVDRLRKYESAYGTQFTPCQLLRDLANNSSKKFYQ
uniprot
proteins
ec_number
1.1.1.211
uniprot
proteins
ec_number
2.3.1.-
uniprot
proteins
ec_number
4.2.1.17
uniprot
proteins
function
FUNCTION: Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through cycles of four consecutive reactions. Each beta-oxidation cycle shortens the fatty acyl-CoA by two carbons, yielding one acetyl-CoA (for the citric acid cycle), one FADH(2), and one NADH (which donate electrons to the respiratory chain for ATP production). These cycles repeat until the chain is fully degraded to acetyl-CoA units. Among the enzymes involved in this pathway, the trifunctional protein--responsible for the hydration, dehydrogenation, and thiolysis steps, shows specificity for long-chain fatty acids, such as those from dietary and stored fats. Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity. These activities have been experimentally confirmed on a few substrates derived from beta-oxidation of long-chain saturated fatty acids such as palmitate (hexadecanoate) and laurate (dodecanoate). In addition, based on its established catalytic mechanism, and combined genetic interaction or mutant phenotype evidence, it is predicted to act also on other substrates, including long-chain unsaturated fatty acids such as oleate (9Z-octadecenoate), linoleate (9Z,12Z-octadecadienoate), linolenate (9Z,12Z,15Z-octadecatrienoate), and others. Independently of subunit beta, HADHA also exhibits a cardiolipin acyltransferase activity that participates in cardiolipin remodeling; cardiolipin is a major mitochondrial membrane phospholipid. HADHA may act downstream of Tafazzin/TAZ, that remodels monolysocardiolipin (MLCL) to a cardiolipin intermediate, and then HADHA may continue to remodel this species into mature tetralinoleoyl-cardiolipin. Has also been proposed to act directly on MLCL; capable of acylating MLCL using different acyl-CoA substrates, with highest activity for oleoyl-CoA. {ECO:0000250|UniProtKB:P40939}.
uniprot
proteins
mass_dalton
82665
uniprot
proteins
pathway_participation
PATHWAY: Lipid metabolism; fatty acid beta-oxidation. {ECO:0000250|UniProtKB:P40939}.
uniprot
proteins
protein_family
Enoyl-CoA hydratase/isomerase family; 3-hydroxyacyl-CoA dehydrogenase family
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
1730633
uniprot
proteins
pubmed
22673903
uniprot
proteins
pubmed
8253773
uniprot
proteins
sequence_length
763
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion {ECO:0000250|UniProtKB:P40939}. Mitochondrion inner membrane {ECO:0000250|UniProtKB:P40939}. Note=Protein stability and association with mitochondrion inner membrane do not require HADHB. {ECO:0000250|UniProtKB:P40939}.
uniprot
proteins