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Article

A variant of the Hspa8 synaptic chaperone modifies disease in a SOD1G86R mouse model of amyotrophic lateral sclerosis.

Experimental neurology - 1 Jan 2025

Takeda Taishi, Her Yoon-Ra, Kim Jeong-Ki, Jha Narendra N, Monani Umrao R

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) is a relatively common and invariably fatal, paralyzing motor neuron disease for which there are few treatment options. ALS is frequently associated with ubiquitin-positive motor neuronal aggregates, a pathology suggestive of perturbed proteostasis. Indeed, cellular chaperones, which are involved in protein trafficking and degradation often underlie familial ALS. Spinal...

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Identifiers

PMID: 39454934
DOI: 10.1016/j.expneurol.2024.115024
PMCID: PMC11611598

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