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Mitochondrial Gene Therapy : An Arena for the Biomedical Use of Inteins

By Owen, Meran, Dr.

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Book Id: WPLBN0000148770
Format Type: PDF eBook
File Size: 0.2 MB
Reproduction Date: 2005
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Title: Mitochondrial Gene Therapy : An Arena for the Biomedical Use of Inteins  
Author: Owen, Meran, Dr.
Language: English
Subject: Health., Medical research, Medical reports
Collections: Medical Library Collection
Publication Date:
Publisher: Centers for Disease Control and Prevention


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Owen, D. M. (n.d.). Mitochondrial Gene Therapy : An Arena for the Biomedical Use of Inteins. Retrieved from

The human mitochondrial DNA (mtDNA) is circular genome of 16 569 bp that encodes 13 proteins and the RNA components of the machinery for their translation (two ribosomal RNAs and 22 transfer RNAs). Because all 13 proteins are subunits of the enzymes that perform oxidative phosphorylation, loss-of-function mutations in any part of the mtDNA deprive the cell of most of its ATP-synthesis capacity. Since 1988, many mtDNA mutations have been discovered that cause neural and/or muscular dysfunction1. Many are inherited, but others occur sporadically and are presumably caused by spontaneous mutations early in development, or even in the unfertilized oocyte.

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