Holocarboxylase synthetase regulation

Gralla, M., Camporeale, G., and Zempleni, J., 2008. Holocarboxylase synthetase regulates expression of biotin transporters by chromatin remodeling events at the SMVT locus. The Journal of Nutritional Biochemistry. 19 400 08. 

MetabolicaUy, biotin is of central importance in lipogenesis, gluconeogen-esis, and the catabolism of branched-chain (and other) amino acids. There are two well-characterized biotin-responsive inborn errors of metabolism, which are fatal if untreated holocarboxylase synthetase deficiency and biotinidase deficiency. In addition, biotin induces a number of enzymes, including glu-cokinase and other key enzymes of glycolysis. Biotinylation of histones may be important in regulation of the cell cycle. 

Rodriguez-Melendez R, Cano S, Mendez ST, and Velazquez A (2001) Biotin regulates the genetic expression of holocarboxylase synthetase and mitochondrial carboxylases in mts. Journal of Nutrition 131, 1909-13. 

Bao, B., Rodriguez-Melendez, R., Wijeratne, S.S., and Zempleni, J., 2010. Biotin regulates the expression of holocarboxylase synthetase in the miR-539 pathway in HEK-293 cells. Journal of Nutrition. 140 1546-1551. 

Solorzano-Vargas, R.S., Pacheco-Alvarez, D., and Leon-Del-Rio, A., 2002. Holocarboxylase synthetase is an obligate participant in biotin-mediated regulation of its own expression and of biotin-dependent carboxylases mRNA levels in human cells. Proceedings of the National Academy of Sciences of the United States of America. 99 5325 5330. 

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