Holocarboxylase synthetase


HLCS is a family of enzymes. This enzyme is important for the effective use of biotin, a B vitamin found in foods such as liver, egg yolks, and milk. In many of the body's tissues, holocarboxylase synthetase activates other specific enzymes 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:
The 3 substrates of this enzyme are ATP, biotin, and, whereas its 3 products are AMP, diphosphate, and.
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 arm of chromosome 21 at position 22.1, from base pair 37,045,059 to base pair 37,284,372.

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