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Identification
HMDB Protein ID HMDBP00048
Secondary Accession Numbers
  • 5277
Name Trifunctional enzyme subunit beta, mitochondrial
Synonyms
  1. 3-ketoacyl-CoA thiolase
  2. Acetyl-CoA acyltransferase
  3. Beta-ketothiolase
  4. TP-beta
Gene Name HADHB
Protein Type Enzyme
Biological Properties
General Function Involved in transferase activity, transferring acyl groups other than amino-acyl groups
Specific Function Not Available
Pathways
  • Carnitine palmitoyl transferase deficiency (I)
  • Carnitine palmitoyl transferase deficiency (II)
  • Ethylmalonic Encephalopathy
  • fatty acid beta-oxidation
  • Fatty acid elongation
  • Fatty Acid Elongation In Mitochondria
  • Fatty acid Metabolism
  • fatty acid metabolism
  • Glutaric Aciduria Type I
  • Long chain acyl-CoA dehydrogenase deficiency (LCAD)
  • Long-chain-3-hydroxyacyl-coa dehydrogenase deficiency (LCHAD)
  • Medium chain acyl-coa dehydrogenase deficiency (MCAD)
  • Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids
  • Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty Acids
  • Short Chain Acyl CoA Dehydrogenase Deficiency (SCAD Deficiency)
  • Trifunctional protein deficiency
  • Valine, leucine and isoleucine degradation
  • Valproic Acid Metabolism Pathway
  • Very-long-chain acyl coa dehydrogenase deficiency (VLCAD)
Reactions
Acyl-CoA + Acetyl-CoA → Coenzyme A + 3-oxoacyl-CoA details
Acyl-CoA + Acetyl-CoA → Coenzyme A + 3-Oxoacyl-CoA details
Succinyl-CoA + Acetyl-CoA → Coenzyme A + 3-Oxoadipyl-CoA details
Propionyl-CoA + Acetyl-CoA → Coenzyme A + 2-Methylacetoacetyl-CoA details
Acetyl-CoA + Butyryl-CoA → Coenzyme A + 3-Oxohexanoyl-CoA details
Octanoyl-CoA + Acetyl-CoA → Coenzyme A + 3-Oxodecanoyl-CoA details
Lauroyl-CoA + Acetyl-CoA → Coenzyme A + 3-Oxotetradecanoyl-CoA details
Tetradecanoyl-CoA + Acetyl-CoA → Coenzyme A + 3-Oxohexadecanoyl-CoA details
Propionyl-CoA + Chenodeoxycholoyl-CoA → Coenzyme A + 3a,7a-Dihydroxy-5b-cholestanoyl-CoA details
Decanoyl-CoA (n-C10:0CoA) + Acetyl-CoA → Coenzyme A + 3-Oxododecanoyl-CoA details
Hexanoyl-CoA + Acetyl-CoA → Coenzyme A + 3-Oxooctanoyl-CoA details
GO Classification
Biological Process
cardiolipin acyl-chain remodeling
glycerophospholipid biosynthetic process
fatty acid beta-oxidation
Cellular Component
endoplasmic reticulum
mitochondrial outer membrane
mitochondrial nucleoid
mitochondrial fatty acid beta-oxidation multienzyme complex
mitochondrial inner membrane
Function
catalytic activity
transferase activity
transferase activity, transferring acyl groups
transferase activity, transferring acyl groups other than amino-acyl groups
Molecular Function
NAD binding
long-chain-enoyl-CoA hydratase activity
fatty-acyl-CoA binding
3-hydroxyacyl-CoA dehydrogenase activity
enoyl-CoA hydratase activity
long-chain-3-hydroxyacyl-CoA dehydrogenase activity
acetyl-CoA C-acyltransferase activity
Process
metabolic process
Cellular Location
  1. Mitochondrion
Gene Properties
Chromosome Location 2
Locus 2p23
SNPs HADHB
Gene Sequence
>1425 bp
ATGACTATCTTGACTTACCCCTTTAAAAATCTTCCCACTGCATCAAAATGGGCCCTCAGA
TTTTCCATAAGACCTCTGAGCTGTTCCTCCCAGCTACGAGCTGCCCCAGCTGTCCAGACC
AAAACGAAGAAGACGTTAGCCAAACCCAATATAAGGAATGTTGTGGTGGTGGATGGTGTT
CGCACTCCATTTTTGCTGTCTGGCACTTCATATAAAGACCTGATGCCACATGATTTGGCT
AGAGCAGCGCTTACGGGTTTGTTGCATCGGACCAGTGTCCCTAAGGAAGTAGTTGATTAT
ATCATCTTTGGTACAGTTATTCAGGAAGTGAAAACAAGCAATGTGGCTAGAGAGGCTGCC
CTTGGAGCTGGCTTCTCTGACAAGACTCCTGCTCACACTGTCACCATGGCTTGTATCTCT
GCCAACCAAGCCATGACCACAGGTGTTGGCTTGATTGCTTCTGGCCAGTGTGATGTGATC
GTGGCAGGTGGTGTTGAGTTGATGTCCGATGTCCCTATTCGTCACTCAAGGAAAATGAGA
AAACTGATGCTTGATCTCAATAAGGCCAAATCTATGGGCCAGCGACTGTCTTTAATCTCT
AAATTCCGATTTAATTTCCTAGCACCTGAGCTCCCTGCGGTTTCTGAGTTCTCCACCAGT
GAGACCATGGGCCACTCTGCAGACCGACTGGCCGCTGCCTTTGCTGTTTCTCGGCTGGAA
CAGGATGAATATGCACTGCGCTCTCACAGTCTAGCCAAGAAGGCACAGGATGAAGGACTC
CTTTCTGATGTGGTACCCTTCAAAGTACCAGGAAAAGATACAGTTACCAAAGATAATGGC
ATCCGTCCTTCCTCACTGGAGCAGATGGCCAAACTAAAACCTGCATTCATCAAGCCCTAC
GGCACAGTGACAGCTGCAAATTCTTCTTTCTTGACTGATGGTGCATCTGCAATGTTAATC
ATGGCGGAGGAAAAGGCTCTGGCCATGGGTTATAAGCCGAAGGCATATTTGAGGGATTTT
ATGTATGTGTCTCAGGATCCAAAAGATCAACTATTACTTGGACCAACATATGCTACTCCA
AAAGTTCTAGAAAAGGCAGGATTGACCATGAATGATATTGATGCTTTTGAATTTCATGAA
GCTTTCTCGGGTCAGATTTTGGCAAATTTTAAAGCCATGGATTCTGATTGGTTTGCAGAA
AACTACATGGGTAGAAAAACCAAGGTTGGATTGCCTCCTTTGGAGAAGTTTAATAACTGG
GGTGGATCTCTGTCCCTGGGACACCCATTTGGAGCCACTGGCTGCAGGTTGGTCATGGCT
GCTGCCAACAGATTACGGAAAGAAGGAGGCCAGTATGGCTTAGTGGCTGCGTGTGCAGCT
GGAGGGCAGGGCCATGCTATGATAGTGGAAGCTTATCCAAAATAA
Protein Properties
Number of Residues 474
Molecular Weight 51293.955
Theoretical pI 9.406
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Trifunctional enzyme subunit beta, mitochondrial
MTILTYPFKNLPTASKWALRFSIRPLSCSSQLRAAPAVQTKTKKTLAKPNIRNVVVVDGV
RTPFLLSGTSYKDLMPHDLARAALTGLLHRTSVPKEVVDYIIFGTVIQEVKTSNVAREAA
LGAGFSDKTPAHTVTMACISANQAMTTGVGLIASGQCDVIVAGGVELMSDVPIRHSRKMR
KLMLDLNKAKSMGQRLSLISKFRFNFLAPELPAVSEFSTSETMGHSADRLAAAFAVSRLE
QDEYALRSHSLAKKAQDEGLLSDVVPFKVPGKDTVTKDNGIRPSSLEQMAKLKPAFIKPY
GTVTAANSSFLTDGASAMLIMAEEKALAMGYKPKAYLRDFMYVSQDPKDQLLLGPTYATP
KVLEKAGLTMNDIDAFEFHEAFSGQILANFKAMDSDWFAENYMGRKTKVGLPPLEKFNNW
GGSLSLGHPFGATGCRLVMAAANRLRKEGGQYGLVAACAAGGQGHAMIVEAYPK
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID P55084
UniProtKB/Swiss-Prot Entry Name ECHB_HUMAN
PDB IDs Not Available
GenBank Gene ID D16481
GeneCard ID HADHB
GenAtlas ID HADHB
HGNC ID HGNC:4803
References
General References
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