New PDF release: Alpha-Keto Acid Dehydrogenase Complexes

By R. N. Perham (auth.), Prof. Mulchand S. Patel, Dr. Thomas E. Roche, Dr. Robert A. Harris (eds.)

ISBN-10: 303488981X

ISBN-13: 9783034889810

ISBN-10: 3034898533

ISBN-13: 9783034898539

Found in all organisms, the alpha-keto acid dehydrogenase complexes have primary roles in mobile metabolism and are significant websites of rules. the knowledge of the association, functionality and law of those indispensable multienzyme complexes has been tremendously complex through experiences making use of molecular biology and biophysical suggestions. even though those enzyme platforms have a few good points in universal, their variety in gratifying designated organism - or tissue - particular roles is actually striking. those platforms have scientific value in components starting from defects in legislation (linked to diabetes, middle sickness, weight problems, foodstuff defects), to inherited illnesses (inborn error, maple syrup urine ailment) to obtained immune ailments (primary biliary cirrhosis). This e-book brings jointly wide-ranging contemporary findings at the structure(function relationships, gene legislation, and genetic defects of the alpha-keto acid dehydrogenase complexes, particularly the pyruvate dehydrogenase, alpha-ketoglutarate dehydrogenase and the branched-chain alpha-keto acid dehydrogenase complexes. a large choice of experimental ways including new effects awarded during this booklet should still function a source for commencing to demonstrated investigators within the box in addition to scientists who're drawn to mitochondria, dehydrogenases, kinases, phosphatases, lipoic acid, thiamine pyrophosphate, and enzyme complexes.

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Biochem. Int. 9: 491-499. S. E. (1991) Investigation of the pyruvate dehydrogenase complex using the circular dichroism method. ) 56: 1840-1849. S. E. (1993) Effect of phosphorylation on the catalytic function of muscular pyruvate dehydrogenase complex. ) 58: 1503-1512. G. S. (1995) Mutagenic studies of the phosphorylation sites of recombinant human pyruvate dehydrogenase. Site-specific regulation. J. Bioi. Chem. 270: 14297 -14304. , Kim, H. S. (1995) Overexpression and characterization of human tetrarneric pyruvate dehydrogenase and its individual subunits.

I. o. (1990) Pea leaf mitochondrial pyruvate dehydrogenase complex is inactivated in vivo in a light-dependent manner. Proc. Natl. Acad. Sci. USA 87: 673-676. F. I. (1977) Binding of thiamin thiazolone pyrophosphate to mammalian pyruvate dehydrogenase and its effects on kinase and phosphatase activities. 1. BioI. Chem. 74: 16671674. M. G. (1994) Investigation of the cofactor-binding site of Zymomonas mobilis pyruvate decarboxylase by site-directed mutagenesis. Biochem. 1. 300: 7 -13. , Clark, B.

157: 4167. G. S. (1991) Sequence conservation in the a and ~ subunits of pyruvate dehydrogenase and its similarity to branched-chain a-keto acid dehydrogenase. FEBS Lett. 282: 209-213. S. S. (1992) A mutation in the Ela subunit of pyruvate dehydrogenase associated with variable expression of pyruvate dehydrogenase complex deficiency. Pediat. Res. 32: 169 - l7 4. , Hartmann, U. A. (1972) Neurospora crassa pyruvate dehydrogenase: interconversion by phosphorylation and dephosphorylation. FEBS Lett.

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Alpha-Keto Acid Dehydrogenase Complexes by R. N. Perham (auth.), Prof. Mulchand S. Patel, Dr. Thomas E. Roche, Dr. Robert A. Harris (eds.)

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