Article
Impaired 26S Proteasome Assembly Precedes Neuronal Loss in Mutant UBQLN2 Rats.
International journal of molecular sciences - 21 Apr 2021
Zhang Wenjuan, Huang Bo, Gao Limo, Huang Cao
Abstract excerpt
Proteasomal dysfunction is known to be associated with amyotrophic lateral sclerosis and frontotemporal degeneration (ALS/FTD). Our previous reports have shown that a mutant form of ubiquilin-2 (UBQLN2) linked to ALS/FTD leads to neurodegeneration accompanied by accumulations of the proteasome subunit Rpt1 in transgenic rats, but the precise pathogenic mechanisms of how this mutation impairs the proteasome...
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