Elsevier S & T

Branched-Chain Amino Acids, Part B

Abelson, John N.; Simon, Melvin I.

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Table of contents

Table of Contentsv
Contributors to Volume 324xi
Prefacexv
Volumes in Seriesxvii
Section I: Preparation of Substrates, Assays of Intermediates and Enzymes, and Use of Enzyme Inhibit1
Chapter 1. Synthesis and Gas Chromatography/Mass Spectrometry Analysis of Stereoisomers of 2-Hydroxy3
Chapter 2. Analysis of Intracellular Metabolites as Tool for Studying Branched-Chain Amino Acid Bios10
Chapter 3. Determination of Branched-Chain L-Amino-Acid Aminotransferase Activity23
Chapter 4. Analysis of (S)- and (R)-3-Methyl-2-oxopentanoate Enantiomorphs in Body Fluids33
Chapter 5. Spectrophotometric Assay for Measuring Branched-Chain Amino Acids40
Chapter 6. Determination of Branched-Chain a-Keto Acid Dehydrogenase Activity State and Branched-Cha48
Chapter 7. Simultaneous Quantification of Plasma Levels of a-Ketoisocaproate and Leucine by Gas Chr62
Chapter 8. Synthesis of Methacrylyl-CoA and (R)- and (S)- Hydroxyisobutyryl-CoA73
Chapter 9. Pathways of Leucine and Valine Catabolism in Yeast80
Section II: Cloning, Expression, and Purification of Enzymes of Branched-Chain Amino Acid Metabolism93
Chapter 10. Isolation of Subunits of Acetohydroxy Acid Synthase Isozyme III and Reconstitution of Ho95
Chapter 11. Branched-Chain Amino-Acid Aminotransferase of Escherichia coli103
Chapter 12. Purification of Sodium-Coupled Branched-Chain Amino Acid Carrier of Pseudomonas aerugino114
Chapter 13. Reconstitution of Pseudomonas aeruginosa High-Affinity Branched-Chain Amino Acid Transpo122
Chapter 14. Purification of Pseudomonas putida Branched-Chain Keto Acid Dehydrogenase E1 Component129
Chapter 15. Pseudomonas mevalonii 3-Hydroxy-3-methylglutaryl-CoA Lyase139
Chapter 16. Human 3-Hydroxy-3-methylglutaryl-CoA Lyase150
Chapter 17. Branched-Chain a-Keto Acid Dehydrogenase Kinase162
Chapter 18. Expression of E1 Component of Human Branched- Chain a-Keto Acid Dehydrogenase Complex in179
Chapter 19. Production of Recombinant Mammalian Holo-E2 and E3 and Reconstitution of Functional Bran192
Chapter 20. Production of Recombinant E1 Component of Branched-Chain a-Keto Acid Dehydrogenase Compl200
Chapter 21. Mammalian Methylmalonate-Semialdehyde Dehydrogenase207
Chapter 22. Mammalian 3-Hydroxyisobutyrate Dehydrogenase218
Chapter 23. 3-Hydroxyisobutyryl-CoA Hydrolase229
Chapter 24. Mammalian Branched-Chain Acyl-CoA Dehydrogenases: Molecular Cloning and Characterization241
Chapter 25. 3-Hydroxy-3-methylglutaryl-CoA Reductase259
Chapter 26. Characterization of 3-Methylcrotonyl-CoA Carboxylase from Plants280
Chapter 27. Purification of D-Hydroxyisovalerate Dehydrogenase from Fusarium sambucinum293
Chapter 28. Purification and Characterization of Recombinant 3-Isopropylmalate Dehydrogenases from T301
Chapter 29. Wild-Type and Hexahistidine-Tagged Derivatives of Leucine-Responsive Regulatory Protein322
Chapter 30. Purification of Branched-Chain Keto Acid Dehydrogenase Regulator from Pseudomonas putida329
Chapter 31. Mitochondrial Import of Mammalian Branched- Chain a-Keto Acid Dehydrogenase Complex Subu336
Chapter 32. Cloning, Expression, and Purification of Mammalian 4-Hydroxyphenylpyruvate Dioxygenase/a342
Chapter 33. Mammalian Branched-Chain Aminotransferases355
Chapter 34. Branched-Chain-Amino-Acid Transaminases of Yeast Saccharomyces cerevisiae365
Chapter 35. Purification, Properties, and Sequencing of Aminoisobutyrate Aminotransferases from Rat376
Chapter 36. Branched-Chain Keto Acid Dehydrogenase of Yeast389
Chapter 37. B-Alanine Synthase, an Enzyme Involved in Catabolism of Uracil and Thymine399
Section III: Detection and Consequences of Genetic Defects in Genes Encoding Enzymes of Branched-Cha411
Chapter 38. Diagnosis and Mutational Analysis of Maple Syrup Urine Disease Using Cell Cultures413
Chapter 39. Detection of Gene Defects in Branched-Chain Amino Acid Metabolism by Tandem Mass Spectro424
Chapter 40. Molecular and Enzymatic Methods for Detection of Genetic Defects in Distal Pathways of B432
Chapter 41. Genetic Defects in E3 Component of a-Keto Acid Dehydrogenase Complexes453
Chapter 42. Targeting E3 Component of a-Keto Acid Dehydrogenase Complexes465
Section IV. Regulation and Expression of Enzymes of Branched-Chain Amino Acid Metabolism477
Chapter 43. Regulation of Expression of Branched-Chain a-Keto Acid Dehydrogenase Subunits in Perman479
Chapter 44. Expression of Murine Branched-Chain a-Keto Acid Dehydrogenase Kinase491
Chapter 45. Regulation of Branched-Chain a-Keto Acid Dehydrogenase Kinase Gene Expression by Glucoco498
Author Index513
Subject Index535

Book details

Vendor Elsevier S & T
SKU 9780121822255
ISBN-13 9780080496795
Author Abelson, John N.; Simon, Melvin I.
Category Science
Subject Organic

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Volume 324 of Methods in Enzymology supplements Volume 166. It includes genetic information (cloning, gene expression) and information on human genetic diseases not available when Volume 166 was published.
General Description of the Series:
The critically acclaimed laboratory standard for more than forty years, Methods in Enzymology is one of the most highly respected publications in the field of biochemistry. Since 1955, each volume has been eagerly awaited, frequently consulted, and praised by researchers and reviewers alike. Now with more than 300 volumes (all of them still in print), the series contains much material still relevant today--truly an essential publication for researchers in all fields of life sciences.

Key Features
* Preparation of substrates and assay of enzymes
* Cloning, expression, and purification of enzymes
* Detection and consequences of genetic defects
* Regulation and expression of enzymes

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