Holocarboxylase synthetase
 | This article is missing information about EC 6.3.4.9, 6.3.4.11, 6.3.4.15. (November 2020) |
| Holocarboxylase synthetase | ||||||
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| RefSeq (mRNA) |
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| RefSeq (protein) |
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| Location (UCSC) | n/a | n/a | ||||
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 | It has been suggested that this article should be HLCS. (discuss ) (November 2020) |
| Biotin—[propionyl-CoA-carboxylase (ATP-hydrolyzing)] ligase | |||||||||
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ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| Gene Ontology | AmiGO / QuickGO | ||||||||
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| Holocarboxylase synthetase (biotin—(propionyl-Coenzyme A-carboxylase (ATP-hydrolysing)) ligase) | |||||||
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| Symbol | HLCS | ||||||
Chr. 21 q22.1 | |||||||
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Holocarboxylase synthetase (biotin—(propionyl-Coenzyme A-carboxylase (ATP-hydrolysing)) ligase)), also known as protein—biotin ligase, is a family of enzymes (
egg yolks, and milk
. In many of the body's tissues, holocarboxylase synthetase activates other specific enzymes (called biotin-dependent carboxylases) by attaching biotin to them. These carboxylases are involved in many critical cellular functions, including the production and breakdown of proteins, fats, and carbohydrates.
The catalyzed reaction:
- ATP + biotin + apo-propionyl-CoA:carbon-dioxide ligase (ADP-forming) AMP + diphosphate + propionyl-CoA:carbon-dioxide ligase (ADP-forming)
The 3
diphosphate, and propionyl-CoA:carbon-dioxide ligase (ADP-forming)
.
Holocarboxylase synthetase may also play a role in regulating the activity of genes. In the nucleus, the enzyme likely attaches biotin molecules to histones, which are structural proteins that bind to DNA and give chromosomes their shape. Changing the shape of histones may help determine whether certain genes are turned on or off; however, it is not known how adding biotin affects gene regulation.
The HLCS gene is located on the long (q) arm of
chromosome 21 at position 22.1, from base pair
37,045,059 to base pair 37,284,372.
Related conditions
carboxylases and histones. Without biotin, carboxylases remain inactive and are unable to process proteins, fats, and carbohydrates
. A lack of holocarboxylase synthetase activity may also alter the regulation of certain genes that are important for normal development. Researchers believe that these disruptions in important cellular functions lead to breathing problems, skin rashes, and the other characteristic signs and symptoms of holocarboxylase synthetase deficiency.
See also
References
- Siegel L, Foote JL, Coon MJ (March 1965). "The enzymatic synthesis of propionyl coenzyme A holocarboxylase from d-biotinyl 5'-adenylate and the apocarboxylase". The Journal of Biological Chemistry. 240 (3): 1025–31. PMID 14284697.
External links
- holocarboxylase+synthetases at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
