Motor neuron apoptosis

Motor neuron apoptosis is a biological process rather than a single molecular target, representing the programmed cell death of motor neurons in neurodegenerative diseases [1, 3]. It is the pathological hallmark of conditions like Amyotrophic Lateral Sclerosis (ALS) and Spinal Muscular Atrophy (SMA), where it leads to progressive muscle weakness and paralysis [3, 9]. The process is triggered by various factors, including glutamate excitotoxicity, oxidative stress, and the accumulation of misfolded proteins such as SOD1 or TDP-43 [1, 8]. These stressors activate the intrinsic and extrinsic apoptotic pathways, involving mediators like the Fas receptor, Bax, and executioner caspases such as Caspase-3 [2, 5, 11]. Therapeutic strategies targeting this process focus on inhibiting pro-apoptotic signaling or correcting genetic defects to prevent neuronal loss [4, 8]. While drugs like Riluzole and Edaravone address upstream mechanisms, direct inhibition of the apoptotic machinery remains a challenge due to the risk of systemic side effects like tumorigenesis [11].

Other names

Programmed cell death of motor neuronsMN apoptosisSpinal motor neuron apoptosis

Inhibition of glutamate excitotoxicity; reduction of oxidative stress; stabilization of mitochondrial function; modulation of RNA processing; inhibition of pro-apoptotic signaling cascades.