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Published online before print November 16, 2005, doi:10.1212/01.wnl.0000188760.53922.05)
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NEUROLOGY 2005;65:1954-1957
© 2005 American Academy of Neurology
Brief Communications

Rapid disease progression correlates with instability of mutant SOD1 in familial ALS

T. Sato, MD*, T. Nakanishi, PhD*, Y. Yamamoto, MD, PhD, P. M. Andersen, MD, PhD, Y. Ogawa, MD, PhD, K. Fukada, MD, PhD, Z. Zhou, MD, PhD, F. Aoike, MD, F. Sugai, MD, S. Nagano, MD, PhD, S. Hirata, MD, M. Ogawa, MD, PhD, R. Nakano, MD, PhD, T. Ohi, MD, PhD, T. Kato, MD, PhD, M. Nakagawa, MD, T. Hamasaki, PhD, A. Shimizu, MD, PhD and S. Sakoda, MD, PhD

From the Department of Neurology (Drs. Sato, Yamamoto, Y. Ogawa, Fukada, Zhou, Aoike, Sugai, Nagano, and Sakoda), Department of Medical Statistics (Dr. Hamasaki), Osaka University Graduate School of Medicine, Suita, Osaka, Japan; Department of Clinical Pathology (Drs. Nakanishi and Shimizu), Osaka Medical College, Takatsuki, Osaka, Japan; Awaji Prefectural Hospital (Dr. Hirata), Sumoto, Hyogo, Japan; Aomori Prefectural Central Hospital (Dr. M. Ogawa), Aomori, Japan; Department of Neurology (Dr. Nakano), Brain Research Institute, Niigata University, Niigata, Japan; Division of Neurology (Dr. Ohi), Department of Internal Medicine, Miyazaki University School of Medicine, Miyazaki, Japan; Third Department of Internal Medicine (Dr. Kato), Yamagata University School of Medicine, Yamagata, Japan; Department of Neurology and Gerontology (Dr. Nakagawa), Research Institute for Neurological Diseases and Geriatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan; and Department of Neurology (Dr. Andersen), Umeå University Hospital, Umeå, Sweden.

Address correspondence and reprint requests to Dr. Yoichi Yamamoto, Department of Neurology D4, Osaka University Graduate School of Medicine, 2-2 Yamada-oka, Suita, Osaka 565-0871, Japan; e-mail: yamamoto{at}neurol.med.osaka-u.ac.jp

Studies on the clinical course of familial ALS suggest that the duration of illness is relatively consistent for each mutation but variable among the different mutations. The authors analyzed the relative amount of mutant compared with normal SOD1 protein in the erythrocytes from 29 patients with ALS with 22 different mutations. Turnover of mutant SOD1 correlated with a shorter disease survival time.

This article was previously published in electronic format as an Expedited E-Pub on November 16, 2005, at www.neurology.org.

* These authors contributed equally to this work.

Supported by Grants-in-Aid for Scientific Research in Japan and by a grant on Specific Diseases (Y.I.) from the Ministry of Health and Welfare, Japan.

Disclosure: The authors report no conflicts of interest.

Received August 4, 2004. Accepted in final form September 1, 2005.




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