Hadhb
entrez
171155
canonical
uniprot
Q60587
canonical
ensembl
ENSRNOG00000010800
resolver
ensembl
ENSRNOG00055020119
resolver
ensembl
ENSRNOG00060011900
resolver
ensembl
ENSRNOG00065024007
resolver
ensp
ENSRNOP00000072480
resolver
ensp
ENSRNOP00055027847
resolver
ensp
ENSRNOP00060015818
resolver
ensp
ENSRNOP00065033671
resolver
enst
ENSRNOT00055034375
resolver
enst
ENSRNOT00060020145
resolver
enst
ENSRNOT00065041223
resolver
genesymbol
Hadhb
resolver
uniprot_entry
ECHB_RAT
resolver
amino_acid_sequence
MTTILTSTFRNLSTTSKWALRFSVRPLSCSSQVQSAPAVQTKSKKTLAKPNLKNIVVVEGVRIPFLLSGTSYKDLMPHDLARAALSGLLYRTNIPKDVVDYIIFGTVIQEVKTSNVAREAALGAGFSDKTPAHTVTMACISSNQAMTTAVGLIASGQCDVVVAGGVELMSDVPIRHSRNMRKMMLDLNKAKTLAQRLSLLTKFRLNFLSPELPAVAEFSTNETMGHSADRLAAAFAVSRMEQDKYALRSHSLAKKAQDEGHLSDIVPFKVPGKDTVSKDNGIRPSSLEQMAKLKPAFIKPYGTVTAANSSFLTDGASAMLIMSEDRALAMGYKPKAYLRDFIYVSQDPKDQLLLGPTYATPKVLEKAGLTMNDIDAFEFHEAFSGQILANFKAMDSDWFAQNYMGRKTKVGAPPLEKFNIWGGSLSLGHPFGATGCRLVMAAANRLRKDGGQYALVAACAAGGQGHAMIVEAYPK
uniprot
proteins
ec_number
2.3.1.16
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 carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB described here 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. {ECO:0000250|UniProtKB:P55084}.
uniprot
proteins
mass_dalton
51414
uniprot
proteins
pathway_participation
PATHWAY: Lipid metabolism; fatty acid beta-oxidation. {ECO:0000250|UniProtKB:P55084}.
uniprot
proteins
protein_family
Thiolase family
uniprot
proteins
protein_family
Thiolase-like superfamily
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
1730633
uniprot
proteins
pubmed
8253773
uniprot
proteins
sequence_length
475
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion {ECO:0000250|UniProtKB:P55084}. Mitochondrion inner membrane {ECO:0000250|UniProtKB:P55084}. Mitochondrion outer membrane {ECO:0000250|UniProtKB:P55084}. Endoplasmic reticulum {ECO:0000250|UniProtKB:P55084}. Note=Protein stability and association with membranes require HADHA. {ECO:0000250|UniProtKB:P55084}.
uniprot
proteins