holocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase)
Symbol
HLCS
(may also be known as: None)
Organism
Human
Description
This gene encodes an enzyme that catalyzes the binding of biotin to carboxylases and histones. The protein plays an important role in gluconeogenesis, fatty acid synthesis and branched chain amino acid catabolism. Defects in this gene are the cause of holocarboxylase synthetase deficiency. Multiple alternatively spliced variants, encoding the same protein, have been identified.[provided by RefSeq, Jun 2011]
Orthologs
| Symbol | Species | |
|---|---|---|
| Hcs | Drosophila melanogaster | |
| hlcs | Danio rerio | |
| Hlcs | Mus musculus | |
| Hlcs | Rattus norvegicus | |
| BPL1 | Saccharomyces cerevisiae | |
| bpl1 | Schizosaccharomyces pombe |
Links to external resources
Changes associated with this gene
GO Terms
| GO ID | GO Term | GO Category | |
|---|---|---|---|
| GO:0006464 | protein modification process | biological_process | |
| GO:0008283 | cell proliferation | biological_process | |
| GO:0009305 | protein biotinylation | biological_process | |
| GO:0016570 | histone modification | biological_process | |
| GO:0070781 | response to biotin | biological_process | |
| GO:0071110 | histone biotinylation | biological_process | |
| GO:0000785 | chromatin | cellular_component | |
| GO:0005625 | soluble fraction | cellular_component | |
| GO:0005652 | nuclear lamina | cellular_component | |
| GO:0005737 | cytoplasm | cellular_component | |
| GO:0005739 | mitochondrion | cellular_component | |
| GO:0005829 | cytosol | cellular_component | |
| GO:0016363 | nuclear matrix | cellular_component | |
| GO:0000166 | nucleotide binding | molecular_function | |
| GO:0004077 | biotin-[acetyl-CoA-carboxylase] ligase activity | molecular_function | |
| GO:0004078 | biotin-[methylcrotonoyl-CoA-carboxylase] ligase activity | molecular_function | |
| GO:0004079 | biotin-[methylmalonyl-CoA-carboxytransferase] ligase activity | molecular_function | |
| GO:0004080 | biotin-[propionyl-CoA-carboxylase (ATP-hydrolyzing)] ligase activity | molecular_function | |
| GO:0005524 | ATP binding | molecular_function | |
| GO:0009374 | biotin binding | molecular_function | |
| GO:0016874 | ligase activity | molecular_function | |
| GO:0018271 | biotin-protein ligase activity | molecular_function | |
| GO:0019899 | enzyme binding | molecular_function | |
| GO:0042803 | protein homodimerization activity | molecular_function | |