Identification
BMDB Protein ID BMDBP00365
Secondary Accession Numbers None
Name Trifunctional enzyme subunit beta, mitochondrial
Synonyms Not Available
Gene Name HADHB
Protein Type Enzyme
Biological Properties
General Function Lipid transport and metabolism
Specific 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 four consecutive reactions breaking down fatty acids into acetyl-CoA. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. 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.
Pathways
  • Fatty acid beta-oxidation
  • Lipid metabolism
Reactions Not Available
GO Classification Not Available
Cellular Location Not Available
Gene Properties
Chromosome Location 11
Locus Not Available
SNPs HADHB
Gene Sequence
>1428 bp
ATGATTTCCCTCTTGACTTACACTTTAAAAAATCTTCCCAATACATCAAAATGGGCCCTC
AGATTTTGTATGAGACCTCTGAGCAGTTCCTCCCAGCTACAAGCTGCCGCAGCTTCCCAG
ACTAAATCGAAAAAAACCCTAGCCAAACCCAACATAAGGAATATTGTGGTGGTGGATGGT
GTTCGCACTCCATTTTTGCTATCAGGCACTTCATATAAAGACCTGATGCCACATGATTTG
GCTAGAGCAGCACTTTCGGGTTTGTTGCATCGGACCAGTGTCCCAAAGGATGTTGTTGAT
TATATCATCTTTGGCACAGTTATACAGGAAGTGAAAACAAGCAATGTGGCTAGAGAGGCT
GCCCTCGGAGCCGGCTTCTCTGACAAGACTCCTGCTCACACTGTCACCATGGCTTGCATC
TCTTCCAACCAAGCCATGACCACAGCTGTCGGCCTGATTGCTTCCGGCCAATGTGACGTG
GTCGTGGCGGGTGGTGTAGAGTTGATGTCTGACATCCCTATTCGCCATTCAAGGAAAATG
AGGAAAATGATGCTGGATCTCAATAAGGCCAAGACTCTGGCTCAGCGACTCTCTATAATC
TCTAAATTCAGATTGAATTTCCTATCGCCAGAGCTTCCTGCAGTGAGTGAGTTCTCCACC
AGTGAGACCATGGGCCACTCTGCAGACCGACTGGCTGCTGCCTTTGCTATTTCTCGGGAG
GAGCAGGATGAATACGCATTGCGCTCTCACAGCCTGGCCAAAAAGGCACAGGATGAAGGA
CTCCTTTCTGATGTTGTGCCCTTCAAAGTACCAGGAAGAGATACAGTTACACAAGATAAT
GGGATTCGTCCTTCCTCTCTGGATCAGATGGCCAAACTAAAACCTGCATTCATCAAGCCC
TATGGCACAGTGACAGCTGCAAATTCTTCTTTCCTGACCGATGGTGCGTCTGCAGTGCTG
ATTATGGCAGAGGAAAAAGCTCTGGCCATGGGTTATAAGCCGAAGGCATATCTGAGGGAT
TTCATGTACGTGTCCCAGGATCCAAAAGATCAGCTTTTACTTGGACCAACGTATGCTACT
CCAAAAGTTCTGGAAAAGGCAGGATTAACAATGAATGATATTGATGTTTTTGAATTTCAT
GAAGCTTTCTCAGGTCAGATTTTGGCTAATTTGAAGGCCATGGATTCTGATTGGTTTGCA
CAAAACTACATGGGTAGAAAAGCCAAGGTTGGATTGCCTCCTTTGGAGAAGTTTAACAAC
TGGGGTGGATCACTGTCTCTGGGGCACCCATTTGGAGCCACTGGCTGTCGGTTGGTCATG
GCAGCTGCCAACAGGTTACGGAAGGAAGGAGGCCAGTATGGTCTAGTAGCTGCCTGTGCA
GCTGGAGGGCAGGGCCATGCTATGATAGTAGAAGCTTATCCAAAATAA
Protein Properties
Number of Residues 475
Molecular Weight 51345.0
Theoretical pI 9.82
Pfam Domain Function Not Available
Signals
  • None
Transmembrane Regions
  • 9-29
  • 82-102
  • 104-122
  • 128-150
Protein Sequence
>Trifunctional enzyme subunit beta, mitochondrial
MISLLTYTLKNLPNTSKWALRFCMRPLSSSSQLQAAAASQTKSKKTLAKPNIRNIVVVDG
VRTPFLLSGTSYKDLMPHDLARAALSGLLHRTSVPKDVVDYIIFGTVIQEVKTSNVAREA
ALGAGFSDKTPAHTVTMACISSNQAMTTAVGLIASGQCDVVVAGGVELMSDIPIRHSRKM
RKMMLDLNKAKTLAQRLSIISKFRLNFLSPELPAVSEFSTSETMGHSADRLAAAFAISRE
EQDEYALRSHSLAKKAQDEGLLSDVVPFKVPGRDTVTQDNGIRPSSLDQMAKLKPAFIKP
YGTVTAANSSFLTDGASAVLIMAEEKALAMGYKPKAYLRDFMYVSQDPKDQLLLGPTYAT
PKVLEKAGLTMNDIDVFEFHEAFSGQILANLKAMDSDWFAQNYMGRKAKVGLPPLEKFNN
WGGSLSLGHPFGATGCRLVMAAANRLRKEGGQYGLVAACAAGGQGHAMIVEAYPK
GenBank ID Protein CAA05840.1
UniProtKB/Swiss-Prot ID O46629
UniProtKB/Swiss-Prot Entry Name ECHB_BOVIN
PDB IDs Not Available
GenBank Gene ID AJ003066
GeneCard ID HADHB
GenAtlas ID Not Available
HGNC ID Not Available
References
General References Not Available