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Joseph disease

Protein patterns in fibroblasts and brain

Laboratory of Cellular Neurobiology, Department of Neurology and Department of Pathology, The University of Texas Health Science Center at Dallas, Dallas, Texas (Drs. Rosenberg, Baskin, and Kirkpatrick and Ms Thomas); and the Department of Pediatrics, University of California, San Diego, San Diego, California (Ms. Bay and Dr. Nyhan).

The separation of brain and fibroblast proteins was analyzed on two-dimensional acrylamide gels. Proteins were examined from skin fibroblast cultures and brain homogenates from the frontal cerebral cortex, putamen, and cerebellum. Protein species from skin fibroblast cultures of controls and patients with Joseph disease or Huntington disease were not significantly different. The proteins from homogenates of the cerebral cortex, putamen, and cerebellum from controls differed from those of one Joseph disease patient. Two major classes of proteins were increased in the patient's putamen and cerebellum. Proteins of 40,000 and 50,000 daltons-including the glial filamentous acidic protein complex (molecular weight 50,000), and two proteins which migrated near actin-were increased in the cerebellum. The glial filamentous acidic protein complex increased 3.7-fold in the putamen of the patient. These protein changes probably represent gliosis, but may also be an expression of the primary genetic mutation.

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