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A Lysosomal Proteinase, the Late Infantile Neuronal Ceroid Lipofuscinosis Gene (CLN2) Product, Is Essential for Degradation of a Hydrophobic Protein, the Subunit c of ATP Synthase

  1. Junji Ezaki,
  2. Isei Tanida,
  3. Nobuo Kanehagi,
  4. Eiki Kominami

Article first published online: 18 JAN 2002

DOI: 10.1046/j.1471-4159.1999.0722573.x

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Journal of Neurochemistry

Journal of Neurochemistry

Volume 72, Issue 6, pages 2573–2582, June 1999

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Ezaki, J., Tanida, I., Kanehagi, N. and Kominami, E. (1999), A Lysosomal Proteinase, the Late Infantile Neuronal Ceroid Lipofuscinosis Gene (CLN2) Product, Is Essential for Degradation of a Hydrophobic Protein, the Subunit c of ATP Synthase. Journal of Neurochemistry, 72: 2573–2582. doi: 10.1046/j.1471-4159.1999.0722573.x

Author Information

  1. Department of Biochemistry, Juntendo University School of Medicine, Tokyo, Japan

* Address correspondence and reprint requests to Dr. E. Kominami at Department of Biochemistry, Juntendo University School of Medicine, Hongo 2-1-1, Bunkyo-ku, Tokyo 113, Japan.

  1. Lippincott Williams & Wilkins, Inc., Philadelphia

  2. Abbreviations used: Cln2p, protein of the CLN2 gene; E-64-d, ethyl(+)-(2S,3S)-3-[(S)-methyl-1-(3-methylbutylcarbamoyl)butylcarbamoyl]-2-oxiranecarboxylate; GST, glutathione S-transferase; INT, 3-(p-iodophenyl)-2-(p-nitrophenyl)-5-phenyl-2H-tetrazolium chloride; LINCL, late infantile form of neuronal ceroid lipofuscinosis; ML fraction, mitochondrial—lysosomal fraction; NCL, neuronal ceroid lipofuscinosis; PAGE, polyacrylamide gel electrophoresis; PBS, phosphate-buffered saline; SDS, sodium dodecyl sulfate.

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  1. Issue published online: 18 JAN 2002
  2. Article first published online: 18 JAN 2002

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Acknowledgements

This work was supported, in part, by a Grant-in Aid for Scientific Research (Intracellular Proteolysis, 08278103 and 10670144) from the Ministry of Education, Science and Culture of Japan, by the Science Research Promotion Fund from the Japan Private School Promotion Foundation. We thank Dr. L. S. Wolfe of the Montreal Neurological Institute for helpful discussions and advice. We also wish to thank Dr. F. Andermann, Dr. S. Carpenter, and Dr. H. Durham (Montreal Neurological Institute), Dr. S. Naidu (Kennedy Krieger Institute, Baltimore, MD, U.S.A.), and Dr. P. Ferreira (Edmonton Genetics Clinic, University of Alberta) for their help in acquiring skin fibroblasts from patients.

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