Cerebellar Hypoplasia in the Gunn Rat with Hereditary Hyperbilirubinemia: Immunohistochemical and Neurochemical Studies

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Abstract
Immunohistochemical reactions were conducted, using the antibodies against GFA [glial fibrillary acidic protein] and S-100 proteins on sections of cerebellum from the homozygous (jj) and the heterozygous (Jj) Gunn rats. Hypertrophy of the fibrous astrocytes was observed but hyperplasia of the glial cells was not. Although the molecular layer was very thin, the Bergmann fiber appeared normal. Among the free amino acids in the cerebellum from the jj rat, glutamate concentration decreased to 2/3 of the control level. The protein profile of the cerebellum from the jj rat obtained by SDS[sodium dodecyl sulfate]-polyacrylamide gel electrophoresis revealed that the amount of P400 protein characteristic of Purkinje cells decreased considerably and there were some changes of the other unidentified proteins. By 2-dimensional electrophoresis of the supernatant from the jj rat cerebellum 1 protein spot diminished and in the particulate fraction from the jj rat 1 spot was enormously increased. The activity of 2'',3''-cyclic nucleotide 3''-phosphohydrolase (CNPase) in the cerebellum from the jj rat did not differ significantly from the control; activities of choline acetyltransferase and acetylcholinesterase of the jj rat were about 2 times as high as those of the control. 2-Deoxyglucose incorporation was maximum in the granular layer from the jj and the Jj rat cerebella. The incorporation in the jj cerebellum was not higher than in the Jj control and even lower in some part of the jj cerebellum than in the control Jj cerebellum.