Inclusions of amyotrophic lateral sclerosis-linked superoxide dismutase in ventral horns, liver, and kidney

Annals of Neurology
P Andreas JonssonStefan L Marklund

Abstract

Mutant superoxide dismutases type 1 (SOD1s) cause amyotrophic lateral sclerosis by an unidentified toxic property. In a patient carrying the G127X truncation mutation, minute amounts of SOD1 were found in ventral horns using a mutant-specific antibody. Still, both absolute levels and ratios versus wild-type SOD1 were considerably greater than in other central nervous system areas and peripheral organs. Inclusions of mutant SOD1 were abundant in motoneurons but were also seen in hepatocytes and kidney epithelium. This first examination of mutant SOD1 in both central nervous system and peripheral organs supports the notion that enrichment of misfolded SOD1s might explain the particular vulnerability of motor areas.

References

Jul 24, 2001·Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders : Official Publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases·S KatoE Ohama
Oct 10, 2003·Brain : a Journal of Neurology·P Andreas JonssonStefan L Marklund
Dec 7, 2005·Brain : a Journal of Neurology·P Andreas JonssonStefan L Marklund
Apr 21, 2006·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Daniel BergemalmStefan L Marklund

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Citations

Nov 26, 2010·Journal of Neurology·Francisco Javier Miana-MenaJoaquín José García
May 21, 2013·Brain : a Journal of Neurology·Rachele A SacconPietro Fratta
May 5, 2012·PloS One·Ann-Sofi JohanssonMikael Oliveberg
Jan 27, 2016·Acta Neuropathologica Communications·Eiichi TokudaStefan L Marklund
Jan 3, 2013·Annals of Neurology·Brandon B Holmes, Marc I Diamond
Apr 12, 2016·Brain Research·Sonam Parakh, Julie D Atkin
Apr 18, 2019·Journal of Neurology, Neurosurgery, and Psychiatry·Karin ForsbergPeter Munch Andersen
Aug 20, 2020·Translational Neurodegeneration·Yoshiaki Furukawa, Eiichi Tokuda
Dec 13, 2016·Cellular and Molecular Neurobiology·Bor Luen Tang
Jul 25, 2019·Brain : a Journal of Neurology·Julien H ParkThorsten Marquardt
Oct 5, 2018·Acta Neuropathologica·Elaheh Ekhtiari BidhendiThomas Brännström

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