Branched-Chain Amino Acids, Part B

Section I: Preparation of Substrates, Assays of Intermediates and Enzymes, and Use of Enzyme Inhibitors

[1]: Synthesis and Gas Chromatography/Mass Spectrometry Analysis of Stereoisomers of 2-Hydroxy-3-methylpentanoic Acid

[2]: Analysis of Intracellular Metabolites as Tool for Studying Branched-Chain Amino Acid Biosynthesis and Its Inhibition in Bacteria

[3]: Determination of Branched-Chain L-Amino-Acid Aminotransferase Activity

[4]: Analysis of (S)- and (R)-3-Methyl-2-oxopentanoate Enantiomorphs in Body Fluids

[5]: Spectrophotometric Assay for Measuring Branched-Chain Amino Acids

[6]: Determination of Branched-Chain a-Keto Acid Dehydrogenase Activity State and Branched-Chain a-Keto Acid Dehydrogenase Kinase Activity and Protein in Mammalian Tissues

[7]: Simultaneous Quantification of Plasma Levels of a-Ketoisocaproate and Leucine by Gas Chromatography–Mass Spectrometry

[8]: Synthesis of Methacrylyl-CoA and (R)- and (S)-3-Hydroxyisobutyryl-CoA

[9]: Pathways of Leucine and Valine Catabolism in Yeast

Section II: Cloning, Expression, and Purification of Enzymes of Branched-Chain Amino Acid Metabolism

[10]: Isolation of Subunits of Acetohydroxy Acid Synthase Isozyme III and Reconstitution of Holoenzyme

[11]: Branched-Chain Amino-Acid Aminotransferase of Escherichia coli

[12]: Purification of Sodium-Coupled Branched-Chain Amino Acid Carrier of Pseudomonas aeruginosa

[13]: Reconstitution of Pseudomonas aeruginosa High-Affinity Branched-Chain Amino Acid Transport System

[14]: Purification of Pseudomonas putida Branched-Chain Keto Acid Dehydrogenase E1 Component

[15]: Pseudomonas mevalonii 3-Hydroxy-3-methylglutaryl-CoA Lyase

[16]: Human 3-Hydroxy-3-methylglutaryl-CoA Lyase

[17]: Branched-Chain a-Keto Acid Dehydrogenase Kinase

[18]: Expression of E1 Component of Human Branched-Chain a-Keto Acid Dehydrogenase Complex in Escherichia coli by Cotransformation with Chaperonins GroEL GroES

[19]: Production of Recombinant Mammalian Holo-E2 and E3 and Reconstitution of Functional Branched-Chain a-Keto Acid Dehydrogenase Complex with Recombinant E1

[20]: Production of Recombinant E1 Component of Branched-Chain a-Keto Acid Dehydrogenase Complex

[21]: Mammalian Methylmalonate-Semialdehyde Dehydrogenase

[22]: Mammalian 3-Hydroxyisobutyrate Dehydrogenase

[23]: 3-Hydroxyisobutyryl-CoA Hydrolase

[24]: Mammalian Branched-Chain Acyl-CoA Dehydrogenases: Molecular Cloning and Characterization of Recombinant Enzymes

[25]: 3-Hydroxy-3-methylglutaryl-CoA Reductase

[26]: Characterization of 3-Methylcrotonyl-CoA Carboxylase from Plants

[27]: Purification of D-Hydroxyisovalerate Dehydrogenase from Fusarium sambucinum

[28]: Purification and Characterization of Recombinant 3-Isopropylmalate Dehydrogenases from Thermus thermophilus Other Microorganisms

[29]: Wild-Type and Hexahistidine-Tagged Derivatives of Leucine-Responsive Regulatory Protein from Escherichia coli

[30]: Purification of Branched-Chain Keto Acid Dehydrogenase Regulator from Pseudomonas putida

[31]: Mitochondrial Import of Mammalian Branched-Chain a-Keto Acid Dehydrogenase Complex Subunits

[32]: Cloning, Expression, and Purification of Mammalian 4-Hydroxyphenylpyruvate Dioxygenase/a-Ketoisocaproate Dioxygenase

[33]: Mammalian Branched-Chain Aminotransferases

[34]: Branched-Chain-Amino-Acid Transaminases of Yeast Saccharomyces cerevisiae

[35]: Purification, Properties, and Sequencing of Aminoisobutyrate Aminotransferases from Rat Liver

[36]: Branched-Chain Keto Acid Dehydrogenase of Yeast

[37]: ß-Alanine Synthase an Enzyme Involved in Catabolism of Uracil and Thymine

Section III: Detection and Consequences of Genetic Defects in Genes Encoding Enzymes of Branched-Chain Amino Acid Metabolism

[38]: Diagnosis and Mutational Analysis of Maple Syrup Urine Disease Using Cell Cultures

[39]: Detection of Gene Defects in Branched-Chain Amino Acid Metabolism by Tandem Mass Spectrometry of Carnitine Esters Produced by Cultured Fibroblasts

[40]: Molecular and Enzymatic Methods for Detection of Genetic Defects in Distal Pathways of Branched-Chain Amino Acid Metabolism

[41]: Genetic Defects in E3 Component of a-Keto Acid Dehydrogenase Complexes

[42]: Targeting E3 Component of a-Keto Acid Dehydrogenase Complexes

Section IV: Regulation and Expression of Enzymes of Branched-Chain Amino Acid Metabolism

[43]: Regulation of Expression of Branched-Chain a-Keto Acid Dehydrogenase Subunits in Permanent Cell Lines

[44]: Expression of Murine Branched-Chain a-Keto Acid Dehydrogenase Kinase

[45]: Regulation of Branched-Chain a-Keto Acid Dehydrogenase Kinase Gene Expression by Glucocorticoids in Hepatoma Cells and Rat Liver

Author Index

Subject Index

• Preparation of substrates and assay of enzymes
• Cloning, expression, and purification of enzymes
• Detection and consequences of genetic defects
• Regulation and expression of enzymes