The Golgi apparatus is fragmented in spinal cord motor neurons of amyotrophic lateral sclerosis with basophilic inclusions

Acta Neuropathologica
Yukio FujitaN K Gonatas

Abstract

The mechanisms of neuronal death in amyotrophic lateral sclerosis (ALS) are not known. A pathological aggregation of cytoplasmic constituents in the form of variety of inclusions may play a role in the pathogenesis of neuronal death. Cytoplasmic basophilic inclusions (BIs) in motor neurons are commonly found in sporadic juvenile ALS. The functional significance of these inclusions is not known, i.e., whether they represent a protective reaction for the isolation of abnormal products from the cytoplasm, or a sign of irreversible neuronal damage. To gain insights on the significance of BIs we asked whether neurons with BIs had an intact or fragmented Golgi apparatus (GA), a sign of neuronal degeneration reported not only in sporadic and familial ALS with mutations of the Cu/Zn superoxide dismutase gene (SOD1), but also in transgenic mice expressing the G93A mutation of SOD1. In these mice fragmentation of the GA of spinal cord motor neurons was found months before the onset of paralysis. We report here that all neurons bearing the inclusions showed fragmentation and reduced number of GA. These results suggest that common pathogenetic mechanisms are involved in the production of BIs and in the fragmentation of the GA.

Citations

Aug 10, 2006·Acta Neuropathologica·Yuji MizunoKoichi Okamoto
Sep 29, 2004·Histochemistry and Cell Biology·Leni Moldovan, Nicanor I Moldovan
Dec 31, 2005·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Yukio Fujita, Koichi Okamoto
Jul 30, 2010·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Yukio FujitaKoichi Okamoto
Feb 18, 2005·NeuroRx : the Journal of the American Society for Experimental NeuroTherapeutics·Merit CudkowiczJeremy Shefner
Jan 19, 2016·Frontiers in Neuroscience·Catherine Rabouille, Georg Haase
Jul 5, 2008·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Jie FanTing Li
Jan 22, 2008·Journal of the Neurological Sciences·Yukio FujitaKoichi Okamoto
Mar 21, 2006·Journal of the Neurological Sciences·Nicholas K GonatasJacqueline O Gonatas
Jun 15, 2010·Neurobiology of Aging·Sivan Vadakkadath Meethal, Craig S Atwood
Oct 30, 2016·Neurobiology of Disease·Alejandro Antón-FernándezAlberto Muñoz
Sep 14, 2007·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Liliana Olim Gouveia, Mamede de Carvalho

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