作者
Benjamin G Trist, Sian Genoud, Stéphane Roudeau, Alexander Rookyard, Amr Abdeen, Veronica Cottam, Dominic J Hare, Melanie White, Jens Altvater, Jennifer A Fifita, Alison Hogan, Natalie Grima, Ian P Blair, Kai Kysenius, Peter J Crouch, Asuncion Carmona, Yann Rufin, Stéphane Claverol, Stijn Van Malderen, Gerald Falkenberg, David J Paterson, Bradley Smith, Claire Troakes, Caroline Vance, Christopher E Shaw, Safa Al-Sarraj, Stuart Cordwell, Glenda Halliday, Richard Ortega, Kay L Double
发表日期
2022/9/1
期刊
Brain
卷号
145
期号
9
页码范围
3108-3130
出版商
Oxford University Press
简介
Aberrant self-assembly and toxicity of wild-type and mutant superoxide dismutase 1 (SOD1) has been widely examined in silico, in vitro and in transgenic animal models of amyotrophic lateral sclerosis. Detailed examination of the protein in disease-affected tissues from amyotrophic lateral sclerosis patients, however, remains scarce.
We used histological, biochemical and analytical techniques to profile alterations to SOD1 protein deposition, subcellular localization, maturation and post-translational modification in post-mortem spinal cord tissues from amyotrophic lateral sclerosis cases and controls. Tissues were dissected into ventral and dorsal spinal cord grey matter to assess the specificity of alterations within regions of motor neuron degeneration.
We provide evidence of the mislocalization and accumulation of structurally disordered, immature SOD1 protein conformers in spinal cord motor …
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BG Trist, S Genoud, S Roudeau, A Rookyard… - Brain, 2022