The pathogenicity scoring system for mitochondrial tRNA mutations revisited

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The pathogenicity scoring system for mitochondrial tRNA mutations revisited

Confirming the pathogenicity of mitochondrial tRNA point mutations is one of the classical challenges in the field of mitochondrial medicine. In addition to genetic and functional studies, the evaluation of a genetic change using a pathogenicity scoring system is extremely useful to discriminate between disease-causing mutations from neutral polymorphisms. The pathogenicity scoring system is ve...

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Mitochondrial DNA Mutations, Pathogenicity and Inheritance

Mitochondria contain their own DNA (mtDNA), which codes for 13 proteins (all subunits of the respiratory chain complexes), 22 tRNAs and 2 rRNAs. Several mtDNA point mutations as well as deletions have been shown to be causative in well-defined mitochondrial disorders. A mixture of mutated and wild type mtDNA (heteroplasmy) is found in most of these disorders. Inheritance of mtDNA is maternal, a...

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ژورنال

عنوان ژورنال: Molecular Genetics & Genomic Medicine

سال: 2013

ISSN: 2324-9269,2324-9269

DOI: 10.1002/mgg3.47