Abstrakt

Amyotrophic lateral sclerosis new molecular pathology pathways.

Sandra Elizabeth

The severe degenerative condition known as Amyotrophic Lateral Sclerosis (ALS) is characterised by the gradual loss of motor neurons in the motor cortex, brainstem, and spinal cord. Frontotemporal lobe dementia can develop along with ALS, despite the latter's classification as a motor condition. ALS starts out focally but spreads to cause paralysis and eventual death. Gene mutations cases and ALS-related genes have been found. Investigations of ALS genes have identified pathogenic roles for: (a) perturbations in protein stability and degradation, (b) altered homeostasis of crucial RNA- and DNA-binding proteins, (c) impaired cytoskeleton function, and (d) non-neuronal cells as modifiers of the ALS phenotype.