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Mitochondrial function and biogenesis / edited by Carla M. Koehler, Matthias F. Bauer. --(Topics in current genetics ; v.8). --Springer, 2004. --(58.1554/M684m)

Contents

Contents

1.Mitochondrial genome evolution: the origin of mitochondria and of eukaryotes  1

2.Mitochondrial biogenesis: protein import into and across the outer membrane  37

3.Mitochondrial biogenesis.protein import into and across the inner membrane  59

4.Mitochondrial tRNA editing  81

5.Protein quality control in mitochondria and neurodegeneration in hereditary spastic paraplegia  97

6.Defects in assembly of cytochrome oxidase: roles in mitochondrial disease  123

7.Function and dysfunction of the oxidative phosphorylation system  149

8.MtDNA maintenance and stabiligy genes: MNGIE and mtDNA depletion syndromes  177

9.Protein translocation into mammalian mitochondria and its role in the development of human mitochondrial disorders  201

10.Mitochondrial fission and fusion machineries  227

11.VDAC function in a cellular context  251

12.Lipid synthesis and transport in mitochondrial biogenesis  267

13.From electron transfer to cholesterol transfer; molecular regulation of steriod synthesis in the mitochondrion  293