Neuromuscular Junction Structural Charactaristics in Familial ALS Mice Model

Abstract

Introduction: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that can cause motor neuron degeneration in the spinal cord and motor cortex. Although most cases of ALS are sporadic, copper-zinc superoxide dismutase-1 (SOD1) mutations are responsible for 10%-20% of familial ALS.
 Objective: Studying neuromuscular junctions in various age groups to see how they vary in structure.
 Methods: From male hemizygous carriers (B6SJL-Tg (SOD1-G93A) 1Gur/J) and female B6SJL/F1 hybrids, female G93ASOD1 transgenic mice and age-matched wild-type (WT littermate partners, both were purchased from Jackson) laboratory. The animals were kept in an environment with controlled humidity and temperature, and 12:12h light was performed in a dark schedule, and sterile rodent food and sterile food were provided without specific pathogens.
 Results: WT and SOD1 are present in 40, 60, 90, 120 days of mice Inside the Tibialis anterior of G93ASOD1 mice (TA) and gastrocnemius (GN) construction. The neurofilament heavy polypeptide (tubulin, green) points to the presynaptic end. α-Bungarotoxins (BTX, white) mark the postsynaptic space, and synaptophysin (p38-1, red) is used for synaptic vesicles. In the following research we demonstrate that in 40 days of mice WT and SOD1 neither of them degraded while in 60 days of mice a slight denaturation occur in WT and SOD1 Further in 90 days of mice the degeneration is not serious about 60% to 70%. Furthermore, the deterioration in mice after 90 days is only 60-70 percent significant.
 Conclusion: NMJ inervation has appeared Disappearance, loss of motor axons, and huge structural damage to the NMJ at 60 and 90 days. Structural changes in presynaptic and postsynaptic structures and a gradual decrease in synaptic vesicles observe.