{Reference Type}: Journal Article
{Title}: Biotin in metabolism, gene expression, and human disease.
{Author}: León-Del-Río A;
{Journal}: J Inherit Metab Dis
{Volume}: 42
{Issue}: 4
{Year}: 07 2019
{Factor}: 4.75
{DOI}: 10.1002/jimd.12073
{Abstract}: Biotin is a water-soluble vitamin that belongs to the vitamin B complex and which is an essential nutrient of all living organisms from bacteria to man. In eukaryotic cells biotin functions as a prosthetic group of enzymes, collectively known as biotin-dependent carboxylases that catalyze key reactions in gluconeogenesis, fatty acid synthesis, and amino acid catabolism. Enzyme-bound biotin acts as a vector to transfer a carboxyl group between donor and acceptor molecules during carboxylation reactions. In recent years, evidence has mounted that biotin also regulates gene expression through a mechanism beyond its role as a prosthetic group of carboxylases. These activities may offer a mechanistic background to a developing literature on the action of biotin in neurological disorders. This review summarizes the role of biotin in activating carboxylases and proposed mechanisms associated with a role in gene expression and in ameliorating neurological disease.