期刊
JOURNAL OF INHERITED METABOLIC DISEASE
卷 42, 期 4, 页码 647-654出版社
WILEY
DOI: 10.1002/jimd.12073
关键词
biotin metabolism; biotinidase deficiency; biotin-responsive disease; biotin-dependent transcription; holocarboxylase synthetase; vitamin disorders
资金
- Programa de Apoyo a Proyectos de Investigacion e Innovacion Tecnologica (PAPIIT), UNAM [IV210218]
- Consejo Nacional de Ciencia y Tecnologia [CB-2014-236405-B]
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.
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