Mouse models of SMA show divergent patterns of neuronal vulnerability and resilience.

ABSTRACT:

Background

Spinal muscular atrophy (SMA) is a form of motor neuron disease affecting primarily children characterised by the loss of lower motor neurons (MNs). Breakdown of the neuromuscular junctions (NMJs) is an early pathological event in SMA. However, not all motor neurons are equally vulnerable, with some populations being lost early in the disease while others remain intact at the disease end-stage. A thorough understanding of the basis of this selective vulnerability will give critical insight into the factors which prohibit pathology in certain motor neuron populations and consequently help identify novel neuroprotective strategies.

Methods

To retrieve a comprehensive understanding of motor neuron susceptibility in SMA, we mapped NMJ pathology in 20 muscles from the

SUBMITTER: Woschitz V 

PROVIDER: S-EPMC9465884 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature