Mitochondrial Function and Biogenesis

by Carla Koehler (Editor), Matthias F. Bauer (Editor)

Overview

Mihondria 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 mihondrial 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 mihondria in different organisms. It particularly focuses on the recent advances in our understanding of basic mihondrial research to the consequences of dysfunction at the molecular level.

The 13 contributions written by leading researchers in the field include topics such as: mihondrial genome evolution and mtDNA stability, mihondrial biogenesis and protein quality control, mihondrial morphology, assembly and function of the mihondrial energy generation apparatus and mihondrial metabolic pathways. These are particularly oriented to link in these various mihondrial pathways to the clinical consequences of their dysfunctions.

Product Details

ISBN-13:	9783540214892
Publisher:	Springer Berlin Heidelberg
Publication date:	06/24/2004
Series:	Topics in Current Genetics , #8
Edition description:	2004
Pages:	333
Product dimensions:	6.10(w) x 9.25(h) x 0.04(d)

Table of Contents

Mihondrial Genome Evolution: The Origin of Mihondria and of Eukaryotes.- Mihondrial Biogenesis: Protein Import into and Across the Outer Membrane.- Mihondrial Biogenesis. Protein Import into and Across the Inner membrane.- Mihondrial tRNA Editing.- Protein Quality Control in Mihondria and Neurodegeneration in Hereditary Spastic Paraplegia.- Defects in Assembly of Cyhrome Oxidase: Roles in Mihondrial Disease.- Function and Dysfunction of the Oxidative Phosphorylation System.- mtDNA Maintenance and Stability Genes: MNGIE and mtDNA Depletion Syndromes.- Protein Translocation into Mammalian Mihondria and Its Role in the Development of Human Mihondrial Disorders.- Mihondrial Fission and Fusion Machineries.- VDAC Function in a Cellular Context.- Lipid Synthesis and Transport in Mihondrial Biogenesis.- From Electron Transfer to Cholesterol Transfer; Molecular Regulation of Steroid Synthesis in the Mihondrion.