LMPD Database

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LMP001519

UniProt Annotations

Entry Information
Gene Nameholocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase)
Protein EntryBPL1_HUMAN
UniProt IDP50747
SpeciesHuman
Comments
Comment typeDescription
Biophysicochemical PropertiesKinetic parameters: KM=224 nM for biotin ; Vmax=143.9 pmol/min/mg enzyme ;
Catalytic ActivityATP + biotin + apo-[3-methylcrotonoyl- CoA
Catalytic ActivityATP + biotin + apo-[acetyl-CoA
Catalytic ActivityATP + biotin + apo-[methylmalonyl-CoA
Catalytic ActivityATP + biotin + apo-[propionyl-CoA
DiseaseHolocarboxylase synthetase deficiency (HLCS deficiency) [MIM
FunctionPost-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl- CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase.
InteractionO00763:ACACB; NbExp=4; IntAct=EBI-3915568, EBI-2211739;
Sequence CautionSequence=AK307940; Type=Frameshift; Positions=169; Evidence= ;
SimilarityBelongs to the biotin--protein ligase family.
SimilarityContains 1 BPL/LPL catalytic domain.
Subcellular LocationCytoplasm. Mitochondrion.
SubunitMonomer.
Tissue SpecificityMostly expressed in muscle, placenta, in lesser extent in the brain, kidney, pancreas, liver and lung.