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 (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.
In the nucleus, the enzyme likely attaches biotin molecules to histones, which are structural proteins that bind to DNA and give chromosomes their shape.
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.