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FLASH GENE
Symbol HLCS contributors: mct - updated : 21-08-2012
HGNC name holocarboxylase synthetase (biotin-(proprionyl-Coenzyme A-carboxylase (ATP-hydrolysing)) ligase)
HGNC id 4976
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • N-terminal domain recognizes the charged region of biotin acceptor protein, distinctly from the recognition by the catalytic domain
  • biotin protein ligase catalytic domain
    • mono polymer monomer
    HOMOLOGY
    interspecies homolog to C.elegans M 162.7
    Homologene
    FAMILY biotin--protein ligase family
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria
    intracellular,cytoplasm,cytosolic
    intracellular,nucleus,nucleoplasm
    text
  • may also enter the nuclear compartment
    • basic FUNCTION
  • catalyzing the biotinylation of the four biotin-dependent carboxylases
  • including the mitochondrial pyruvate carboxylase, propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase and the cytosolic acetyl-CoA carboxylase
  • catalyzes the incorporation of biotin into apo carboxylase and the biotinylation of the four biotin-dependent carboxylases in the cell
  • responsible for attaching biotin onto the biotin-dependent enzymes that reside in the cytoplasm and mitochondria
  • plays a pivotal role in biotin-dependent metabolic and epigenetic phenomena
  • biotin protein ligase, which has a pivotal role in biotin-dependent metabolic pathways and epigenetic phenomena
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interacts directly with histone H3, causing biotinylation of K9 and K18
  • HLCS acts as a biotin-independent transcriptional repressor interacting with HDAC1, HDAC2 and HDAC7
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) HLCS
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS