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- W291233445 abstract "This chapter focuses on mitochondrial genes and neurological disease. Mitochondria are cylindrical structures with a diameter of 0.5–1.0 μm. They consist of an outer membrane, an inner membrane, and two internal compartments, the intermembranous space and the matrix. The nucleus is not the only organelle containing DNA in mammalian cells; mitochondria possess their own independently replicating genetic system. Each mammalian mitochondrion contains five to ten circular DNA molecules, which are double-stranded and 16.6 kilobases (kb) in length. Mitochondrial (mt) DNA differs from nuclear DNA in that it contains very little noncoding sequence and to some extent in its genetic code, which dictates, for example, that UGA reads tryptophan instead of a stop codon. The chapter discusses mitochondrial myopathy (MM), a term applied to a clinically and biochemically heterogeneous group of disorders, which share the common feature of major mitochondrial structural abnormalities in skeletal muscle. Ragged red fibers containing peripheral and intermyofibrillar accumulations of abnormal mitochondria, seen with the modified Gomori trichrome stain, are the major morphological hallmark of these diseases." @default.
- W291233445 created "2016-06-24" @default.
- W291233445 creator A5081825582 @default.
- W291233445 date "1988-01-01" @default.
- W291233445 modified "2023-09-25" @default.
- W291233445 title "Mitochondrial genes and neurological disease" @default.
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- W291233445 doi "https://doi.org/10.1016/b978-0-407-02400-7.50020-2" @default.
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