Endplate denervation correlates with Nogo-A muscle expression in amyotrophic lateral sclerosis patients.

ABSTRACT:

Objective

Data from mouse models of amyotrophic lateral sclerosis (ALS) suggest early morphological changes in neuromuscular junctions (NMJs), with loss of nerve-muscle contact. Overexpression of the neurite outgrowth inhibitor Nogo-A in muscle may play a role in this loss of endplate innervation.

Methods

We used confocal and electron microscopy to study the structure of the NMJs in muscle samples collected from nine ALS patients (five early-stage patients and four long-term survivors). We correlated the morphological results with clinical and electrophysiological data, and with Nogo-A muscle expression level.

Results

Surface electromyography assessment of neuromuscular transmission was abnormal in 3/9 ALS patients. The postsynaptic apparatus was morphologically alter

SUBMITTER: Bruneteau G 

PROVIDER: S-EPMC4402082 | biostudies-literature | 2015 Apr

REPOSITORIES: biostudies-literature