Muscle cells of sporadic amyotrophic lateral sclerosis patients secrete neurotoxic vesicles.

ABSTRACT:

Background

The cause of the motor neuron (MN) death that drives terminal pathology in amyotrophic lateral sclerosis (ALS) remains unknown, and it is thought that the cellular environment of the MN may play a key role in MN survival. Several lines of evidence implicate vesicles in ALS, including that extracellular vesicles may carry toxic elements from astrocytes towards MNs, and that pathological proteins have been identified in circulating extracellular vesicles of sporadic ALS patients. Because MN degeneration at the neuromuscular junction is a feature of ALS, and muscle is a vesicle-secretory tissue, we hypothesized that muscle vesicles may be involved in ALS pathology.

Methods

Sporadic ALS patients were confirmed to be ALS according to El Escorial criteria and were genot

SUBMITTER: Le Gall L 

PROVIDER: S-EPMC8978001 | biostudies-literature | 2022 Apr

REPOSITORIES: biostudies-literature