PMID: 2511498Sep 1, 1989Paper

A comparative immunocytochemical study of human cerebellar cortex in X-chromosome-linked copper malabsorption (Menkes' kinky hair disease) and granule cell type cerebellar degeneration

Neuropathology and Applied Neurobiology
S GotoR R Rojas-Corona

Abstract

A comparative immunocytochemical study on the cerebellar cortex with X-chromosome-linked copper malabsorption (X-cLCM) and granule cell type cerebellar degeneration (gc-CD) was carried out by using specific monoclonal antibodies to synaptophysin (SY) and glial fibrillary acidic protein (GFAP). In X-cLCM cases, marked depletion of SY-immunoreactivity (IR) and reduction in number of SY-positive glomeruli were seen in the molecular and granular layers, respectively. Abnormal Purkinje cells occasionally showed moderately strong SY-IR having a fine granular pattern. Proliferation of GFAP-positive cells was observed in the granular and Purkinje cell layers. In the gc-CD case, SY-positive materials were coarsely distributed in a less dense fashion in the molecular layer as compared to a normal control. Purkinje cell perikarya did not show SY-IR. In the gc-CD granular layer, SY-IR appeared to have a coarsely punctate pattern, whereas immunoreactive glomeruli were almost completely absent. A number of GFAP-positive Bergmann cells was observed in the Purkinje cell layer and their fibres were densely and irregularly distributed in the molecular layer, whereas the granular layer was devoid of GFAP-positive cells. We present an immunocytoch...Continue Reading

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Citations

Jan 1, 1990·Acta Neuropathologica·S Goto, A Hirano
Mar 1, 1996·Journal of the Neurological Sciences·A IkemotoJ Kimura
Jul 23, 2005·The Cerebellum·Kea Joo LeeIm Joo Rhyu
Aug 1, 1990·Neuropathology and Applied Neurobiology·S Goto, A Hirano
Sep 7, 1999·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·J H Menkes
Jan 23, 2018·Translational Science of Rare Diseases·Carlos R Ferreira, William A Gahl
Mar 1, 1996·The American Journal of Surgical Pathology·P J Zhang, M K Rosenblum

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