Implications of Selective Autophagy Dysfunction for ALS Pathology.

Abstract:

:Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disorder that progressively affects motor neurons in the brain and spinal cord. Due to the biological complexity of the disease, its etiology remains unknown. Several cellular mechanisms involved in the neurodegenerative process in ALS have been found, including the loss of RNA and protein homeostasis, as well as mitochondrial dysfunction. Insoluble protein aggregates, damaged mitochondria, and stress granules, which contain RNA and protein components, are recognized and degraded by the autophagy machinery in a process known as selective autophagy. Autophagy is a highly dynamic process whose dysregulation has now been associated with neurodegenerative diseases, including ALS, by numerous studies. In ALS, the autophagy process has been found deregulated in both familial and sporadic cases of the disease. Likewise, mutations in genes coding for proteins involved in the autophagy machinery have been reported in ALS patients, including selective autophagy receptors. In this review, we focus on the role of selective autophagy in ALS pathology.

journal_name

Cells

journal_title

Cells

authors

Vicencio E,Beltrán S,Labrador L,Manque P,Nassif M,Woehlbier U

doi

10.3390/cells9020381

subject

Has Abstract

pub_date

2020-02-07 00:00:00

issue

2

issn

2073-4409

pii

cells9020381

journal_volume

9

pub_type

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