Amyotrophic Lateral Sclerosis (ALS) is a complex neurodegenerative disorder characterized by progressive axonopathy, jointly leading to the dying back of the motor neuron, disrupting both nerve signaling and motor control. In this review, we highlight the roles of axonopathy in ALS progression, driven by the interplay of multiple factors including defective trafficking machinery, protein aggregation, and mitochondrial dysfunction. Dysfunctional intracellular transport, caused by disruptions in microtubules, molecular motors, and adaptors, has been identified as a key contributor to disease progression. Aberrant protein aggregation involving TDP-43, FUS, SOD1, and dipeptide repeat proteins further amplifies neuronal toxicity. Mitochondrial defects lead to ATP depletion, oxidative stress, and Ca²⁺ imbalance, which are regarded as key factors underlying the loss of neuromuscular junctions and axonopathy. Mitigating these defects through interventions including neurotrophic treatments offers therapeutic potential. Collaborative research efforts aim to unravel ALS complexities, opening avenues for holistic interventions that target diverse pathological mechanisms.
Humans ; Amyotrophic Lateral Sclerosis/therapy* ; Animals ; Axons/metabolism* ; Mitochondria/metabolism* ; Motor Neurons/pathology*

We tested the mechanical properties of fiber layers in adult anulus fibrosus of lumbar interverbral disc 4,5. Aulus fibrosus of lumbar interverbral disc was delaminated by using microsurgical technique. 8 testing points were taken in each layer according to the fibers going and 5 testing specimens were taken from each testing point. The length was 15-20mm, the width 1-2mm, and the thickness 0. 1-0. 5mm. By using tension test,the relationship curves of stress and strain were tested, the fitting curves and equations were obtained, and then the elastic modulus, damage strain and damage stress were obtained. Therefore we knew the parameters and equations of mechanical properties of each testing point. We got the result that the elastic modulus of each testing point, along the radius from outside to inside, decreased with the increase of layers. The damage stress decreased linearly from outside to inside. The damage strains of the outside 9 layers increased slightly with the increase of layers, and the others were almost constants, i. e. 0.34 +/- 0.14. We get the conclusion that analus fibrosus of intervertebral disc has special mechanical properties corresponding with its functions, which is in a close relationship with lumbar vertebral diseases.
Adult ; Biomechanical Phenomena ; Elasticity ; Humans ; Intervertebral Disc ; physiology ; Lumbar Vertebrae ; physiology ; Male ; Stress, Mechanical ; Tensile Strength ; physiology