Applications - SDH lab

Applications - SDH lab - Rustin, P., Munnich, A., Rotig, A....

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Rustin, P., Munnich, A., Rotig, A. (2002) Succinate dehydrogenase and human diseases: new insights into a well-known enzyme. European Journal of Human Genetics, 10 , 289-91. Pancrudo, J., Shanske, S., Bonilla, E., Daras, M., Akman, H., Krishna, S., Malkin, E. (2007) Mitochondrial encephalomyopathy due to a novel mutation in the tRNAGlu of Mitochondrial DNA. J. Child Neurol , 22 , 858-62. Gottlieb, E., Tomlinson, I. (2005) Mitochondrial Tumor Suppressors: a Genetic and Biochemical Update. Medscape , 22 , 834. Milakovic, T., Johnson, G. (2005) Mitochondrial Respiration and ATP Production are Significantly Impaired in Striatal Cells Expressing Mutant Huntingtin. J. Biol. Chem, 280 , 30773-30782. Applications : Malonate is a competitive inhibitor of cellular respiration because it binds to the active site of succinate dehydrogenase in the tricarboxylic acid cycle. However, because it lacks the functional dimethyl group that is present in succinate, dehydrogenation cannot occur and it cannot become fumarate. Also, malonate is an inhibitor for complex II in the respiratory chain.
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This note was uploaded on 05/21/2008 for the course BIO 215 taught by Professor Duwel during the Winter '08 term at Drexel.

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