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  • Title: Two proteins associated with cerebellar hypoplasia in jaundiced Gunn rats.
    Author: Aono S, Sato H, Semba R, Kashiwamata S.
    Journal: Neurochem Res; 1983 Jun; 8(6):743-56. PubMed ID: 6621772.
    Abstract:
    Two cerebellar proteins with apparent molecular weights of 250,000 (GR-250) and 50,000 (GR-50) are closely associated with cerebellar hypoplasia in jaundiced homozygous Gunn rats. These proteins, found in Gunn rat cerebellum (4-60 days of age) and cerebrum as well as staggerer mouse cerebellum, were studied with electrophoretic techniques. After 8 days of life, GR-250 decreased and GR-50 increased in the homozygous Gunn rat cerebellum. The pI's of GR-250 and GR-50 were 4.7-5.8 and 4.6-4.9, respectively, and the former protein was shown to bind to Concanavalin A. A comparative study between cerebella of Gunn rats and staggerer mice revealed that GR-250 and P400, a protein generally thought to be characteristic of the Purkinje cells, were identical. Evidence was also obtained showing that GR-250 was present in the Gunn rat cerebrum. GR-50 was not detectable in the staggerer mouse cerebellum but instead, a protein (MW 47,000) was found to be increased in the mutant mouse cerebellum.
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