PMID: 9535127Apr 16, 1998Paper

A study of cell death in Werdnig Hoffmann disease brain

Neuroscience Letters
M HayashiH Matsuyama

Abstract

We examined the occurrence of apoptotic cell death in the autopsied brains of four patients with Werdnig Hoffmann disease (WH), using TdT-mediated DIG-dUTP nick end labeling (TUNEL) and immunohistochemistry for apoptosis-related proteins. Three of the four patients, aged over 6 months, exhibited TUNEL-positive cells in the lateral nuclei of the thalamus, and one of the three patients also had TUNEL-positive cells in the cerebral cortex. The labeled nuclei did not show characteristic features such as nuclear fragmentation or apoptotic bodies, and synaptophysin-positive granules were observed around some of the TUNEL-positive cells, although none of the antibodies against glial markers could visualize TUNEL-positive cells. TUNEL-positive cells were not observed in other regions examined, including the spinal cord, medulla and cerebellum or in the brains of three age-matched controls. There were neither immunopositive structures for bcl-2 or p53 nor alteration of in situ expression of bcl-xs/l or bax in any subject, and the TUNEL-positive cells lacked immunopositivity against apoptosis-related proteins. The presence of these TUNEL-positive cells might suggest latent neurodegeneration in the thalamus before central chromatolysis of...Continue Reading

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Citations

Feb 9, 2006·Acta Neuropathologica·Yasuo HachiyaKiyoko Kurata
Nov 28, 2002·Brain & Development·Masaharu HayashiYoshio Morimatsu
Aug 14, 2010·Human Molecular Genetics·Thomas M WishartThomas H Gillingwater
Jun 29, 2011·Disease Models & Mechanisms·James N SleighKevin Talbot
Jul 8, 2010·Brain & Development·Yasushi ItoMakiko Osawa
Apr 17, 2001·Brain Pathology·H Schmalbruch, G Haase
Jul 11, 2000·Journal of Neuropathology and Experimental Neurology·G SimicB Kruslin

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