About the Book
Part I: Mitochondrial Disorder: A Complex Disease of the Two Genomes
1. Mitochondrial DNA Mutations: An Overview of Clinical and Molecular Aspects
William J. Craigen
2. Nuclear Gene Defects in Mitochondrial Disorders
Fernando Scaglia
19. Sequence Analysis of the Whole Mitochondrial Genome and Nuclear Genes Causing Mitochondrial Disorders
3. Diagnostic Challenges of Mitochondrial Disorders: Complexities of Two Genomes
Brett H. Graham
Part II: Biochemical Analysis of Mitochondrial Disorders
4. Biochemical Analyses of the Electron Transport Chain Complexes by Spectrophotometry
19. Sequence Analysis of the Whole Mitochondrial Genome and Nuclear Genes Causing Mitochondrial Disorders
Ann E. Frazier and David R. Thorburn
5. Measurement of Mitochondrial Oxygen Consumption Using A Clark Electrode
Zhihong Li and Brett H. Graham
6. Mitochondrial Respiratory Chain: Biochemical Analysis and Criterion for Deficiency in Diagnosis
19. Sequence Analysis of the Whole Mitochondrial Genome and Nuclear Genes Causing Mitochondrial Disorders
Manuela M. Grazina
7. Assays of Pyruvate Dehydrogenase Complex and Pyruvate Carboxylase Activity
19. Sequence Analysis of the Whole Mitochondrial Genome and Nuclear Genes Causing Mitochondrial Disorders
Douglas Kerr, George Grahame, and Ghunwa Nakouzi
8. Assessment of Thymidine Phosphorylase Function: Measurement of Plasma Thymidine (and Deoxyuridine) and Thymidine Phosphosphorylase Activity
19. Sequence Analysis of the Whole Mitochondrial Genome and Nuclear Genes Causing Mitochondrial Disorders
Ramon Martí, Luis C. López, and Michio Hirano
9. Measurement of Mitochondrial dNTP Pools
19. Sequence Analysis of the Whole Mitochondrial Genome and Nuclear Genes Causing Mitochondrial Disorders
Ramon Martí, Beatriz Dorado, and Michio Hirano
10. Measurement of Oxidized and Reduced Coenzyme Q in Biological Fluids, Cells, and Tissues: An HPLC-EC Method
19. Sequence Analysis of the Whole Mitochondrial Genome and Nuclear Genes Causing Mitochondrial Disorders
Peter H. Tang and Michael V. Miles
11. Assay to Measure Oxidized and Reduced Forms of CoQ by LC-MS/MS
19. Sequence Analysis of the Whole Mitochondrial Genome and Nuclear Genes Causing Mitochondrial Disorders
Si Houn Hahn, Sandra Kerfoot, and Valeria Vasta
12. Morphological Assessment of Mitochondrial Respiratory Chain Function on Tissue Sections
19. Sequence Analysis of the Whole Mitochondrial Genome and Nuclear Genes Causing Mitochondrial Disorders
Kurenai Tanji
13. Blue Native Polyacrylamide Gel Electrophoresis: A Powerful Diagnostic Tool for the Detection of Assembly Defects in the Enzyme Complexes of Oxidative Phosphorylation
19. Sequence Analysis of the Whole Mitochondrial Genome and Nuclear Genes Causing Mitochondrial Disorders
Scot C. Leary
14. Radioactive Labeling of Mitochondrial Translation Products in Cultured Cells
19. Sequence Analysis of the Whole Mitochondrial Genome and Nuclear Genes Causing Mitochondrial Disorders
Florin Sasarman and Eric A. Shoubridge
15. Transmitochondrial Cybrids:
