Beta Amyloid Peptide: Research Paper : Lessons learned from CHMP2B, implications for frontotemporal dementia and amyotrophic lateral sclerosis

Lessons learned from CHMP2B, implications for frontotemporal dementia and amyotrophic lateral sclerosis

Abstract

Frontotemporal dementia (FTD) and Amyotrophic Lateral Sclerosis (ALS) are two neurodegenerative diseases with clinical, genetic and pathological overlap. As such, they are commonly regarded as a single spectrum disorder, with pure FTD and pure ALS representing distinct ends of a continuum. Dysfunctional endo-lysosomal and autophagic trafficking, leading to impaired proteostasis is common across the FTD-ALS spectrum. These pathways are, in part, mediated by CHMP2B, a protein that coordinates membrane scission events as a core component of the ESCRT machinery. Here we review how ALS and FTD disease causing mutations in CHMP2B have greatly contributed to our understanding of how endosomal-lysosomal and autophagic dysfunction contribute to neurodegeneration, and how in vitro and in vivo models have helped elucidate novel candidates for potential therapeutic intervention with implications across the FTD-ALS spectrum.

Keywords: ALS; CHMP2B; ESCRT; FTD; Immunity; Motor neurone disease; Neurodegeneration; Proteostasis; Therapeutics.

This article originally appeared in the "https://pubmed.ncbi.nlm.nih.gov/33144171/" and has their copyrights. We do not claim copyright on the content. This information is for research purposes only. This Blog is made available by publishers for educational purposes only as well as to give you general information and a general understanding , not to provide specific advice. By using this blog site you understand that there is no client relationship between you and the Blog publisher. The Blog should not be used as a substitute for competent research advice.