Question

Can someone show the step by step biochemical mechanism for ALS disease and explain each step?

Answer 1

ALS, amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Myo means muscle and trophic means nourishment; therefore amyotrophic means lack of muscle nourishment. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates, it leads to hardening (sclerosis) in the region.

ALS kills motor neurons and causes weakening of muscles. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, people may lose the ability to speak, eat, move and breathe.

Commonly proposed pathogenic mechanisms include RNA metabolism and protein metabolism. Oxidative stress also induces the pathogenesis of ALS. Mitochondrial dysfunction is a common feature of many neurodegenerative disorders.

Glutamate excitotoxicity has long been implicated in ALS pathogenesis.. In ALS, there is an understandable focus on the motor neurons, but evidence exists that this is a non-cell-autonomous disease, and non-neuronal cells, in particular microglia may have an important role in pathogenesis.