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Uniform tissue distribution of tRNA^Lys mutation in mitochondrial DNA in MERRF patients

Department of Internal Medicine (Dr. Tanno), National Sanatorium Nishi-Ojiya Hospital, Ojiya; the Departments of Neurology (Drs. Tanno, Yoneda, Tanaka, Kondo, Hozumi. and Tsuji) and Pathology (Drs. Wakabayashi, Yamada, and Ikuta), Brain Research Institute, Niigata University, Niigata; and the Department of Neurology (Dr. Fukuhara), National Saigata Hospital, Oogata-machi, Japan.

We documented the presence of a point mutation in the tRNA^Lys gene of mitochondrial DNA (mtDNA) in various postmortem tissues from two patients with myoclonus epilepsy associated with ragged-red fibers (MERRF). The percentages of the mutant mtDNA were similar (93 to 99%) in both clinically affected and unaffected tissues, suggesting that preferential clinical involvement of certain tissues in MERRF is based not only on the variation of distribution of the mutant mtDNA, but also on other factors such as differences in the threshold in various CNS regions and organs.

Address correspondence and reprint requests to Dr. Y. Tanno, Department of Neurology, Brain Research Institute, Niigata University, 1 Asahimachi, Niigata 951, Japan.

Supported in part by a Grant-in-Aid for Scientific Research on Priority Area, Ministry of Education, Science and Culture, and Grant (2A-2) from the National Center of Neurology and Psychiatry (NCNP) of the Ministry of Health and Welfare, Japan.

Received June 19, 1992. Accepted for publication in final form October 1,1992.

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