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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
EXPRESSION
Type
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestiveliver   moderately
Endocrinepancreas   moderately
Nervousbrain   moderately
Respiratorylung   moderately
Urinarykidney   moderately
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Epithelialsecretoryglandularendocrine 
Epithelialsecretoryglandularexocrine 
Muscularstriatumskeletal  
cell lineage
cell lines
fluid/secretion
at STAGE
physiological period pregnancy
Text placenta
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