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trifunctional enzyme beta subunit, mitochondrial; TP-beta; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase beta subunit (HADHB)
Function:
- lipid metabolism; fatty acid beta-oxidation
- octamer of 4 alpha (HADHA) & 4 beta (HADHB) subunits
- interacts with RSAD2/viperin [2]
Structure: belongs to the thiolase family
Compartment:
- mitochondria
- endoplasmic reticulum
Alternative splicing: named isoforms=2
Pathology:
- defects in HADHB are a component of trifunctional protein deficiency [3]
Related
HADHB gene mutation
General
dehydrogenase
mitochondrial protein
protein subunit
Properties
SIZE: MW = 51 kD
entity length = 474 aa
COMPARTMENT: mitochondria
endoplasmic reticulum
MOTIF: cysteine residue {C138}
histidine residue {H428}
cysteine residue {C458}
Database Correlations
OMIM correlations
UniProt P55084
PFAM correlations
Entrez Gene 3032
KEGG correlations
ENZYME 2.3.1.16
References
- UniProt :accession P55084
- PMID: 8163672
- PMID: 9259266
Component-of
mitochondrial trifunctional enzyme complex (trifunctional protein complex, TFP complex, trifunctional enzyme)