Ebook: Mitochondrial Function and Biogenesis
- Tags: Biochemistry general, Cell Biology, Human Genetics
- Series: Topics in Current Genetics 8
- Year: 2004
- Publisher: Springer-Verlag Berlin Heidelberg
- Edition: 1
- Language: English
- pdf
Mitochondria are complex organelles, possessing a double-membrane and even their own genome, the mtDNA. They play a pivotal role in cellular metabolism, respiration, and production of ATP essential for the normal function of all human organ systems. It is not surprising, therefore, that genetic defects of mitochondrial functions cause a wide spectrum of human diseases. This book provides the first modern and truly comprehensive coverage of the biochemistry, genetics, and pathology of mitochondria in different organisms. It particularly focuses on the recent advances in our understanding of basic mitochondrial research to the consequences of dysfunction at the molecular level.
The 13 contributions written by leading researchers in the field include topics such as: mitochondrial genome evolution and mtDNA stability, mitochondrial biogenesis and protein quality control, mitochondrial morphology, assembly and function of the mitochondrial energy generation apparatus and mitochondrial metabolic pathways. These are particularly oriented to link in these various mitochondrial pathways to the clinical consequences of their dysfunctions.
Mitochondria are complex organelles, possessing a double-membrane and even their own genome, the mtDNA. They play a pivotal role in cellular metabolism, respiration, and production of ATP essential for the normal function of all human organ systems. It is not surprising, therefore, that genetic defects of mitochondrial functions cause a wide spectrum of human diseases. This book provides the first modern and truly comprehensive coverage of the biochemistry, genetics, and pathology of mitochondria in different organisms. It particularly focuses on the recent advances in our understanding of basic mitochondrial research to the consequences of dysfunction at the molecular level.
The 13 contributions written by leading researchers in the field include topics such as: mitochondrial genome evolution and mtDNA stability, mitochondrial biogenesis and protein quality control, mitochondrial morphology, assembly and function of the mitochondrial energy generation apparatus and mitochondrial metabolic pathways. These are particularly oriented to link in these various mitochondrial pathways to the clinical consequences of their dysfunctions.
Mitochondria are complex organelles, possessing a double-membrane and even their own genome, the mtDNA. They play a pivotal role in cellular metabolism, respiration, and production of ATP essential for the normal function of all human organ systems. It is not surprising, therefore, that genetic defects of mitochondrial functions cause a wide spectrum of human diseases. This book provides the first modern and truly comprehensive coverage of the biochemistry, genetics, and pathology of mitochondria in different organisms. It particularly focuses on the recent advances in our understanding of basic mitochondrial research to the consequences of dysfunction at the molecular level.
The 13 contributions written by leading researchers in the field include topics such as: mitochondrial genome evolution and mtDNA stability, mitochondrial biogenesis and protein quality control, mitochondrial morphology, assembly and function of the mitochondrial energy generation apparatus and mitochondrial metabolic pathways. These are particularly oriented to link in these various mitochondrial pathways to the clinical consequences of their dysfunctions.
Content:
Mitochondrial genome evolution: the origin of mitochondria and of eukaryotes....Pages 1-35
Mitochondrial biogenesis: Protein import into and across the outer membrane....Pages 37-58
Mitochondrial biogenesis. Protein import into and across the inner membrane....Pages 59-80
Mitochondrial tRNA editing....Pages 81-96
Protein quality control in mitochondria and neurodegeneration in hereditary spastic paraplegia....Pages 97-121
Defects in assembly of cytochrome oxidase: roles in mitochondrial disease....Pages 123-148
Function and dysfunction of the oxidative phosphorylation system....Pages 149-176
MtDNA maintenance and stability genes: MNGIE and mtDNA depletion syndromes....Pages 177-200
Protein translocation into mammalian mitochondria and its role in the development of human mitochondrial disorders....Pages 201-225
Mitochondrial fission and fusion machineries....Pages 227-249
VDAC function in a cellular context....Pages 251-266
Lipid synthesis and transport in mitochondrial biogenesis....Pages 267-291
From electron transfer to cholesterol transfer; molecular regulation of steroid synthesis in the mitochondrion....Pages 293-330