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Fig. 1 | Translational Neurodegeneration

Fig. 1

From: Environmental insults: critical triggers for amyotrophic lateral sclerosis

Fig. 1

Proposed model of ALS pathogenesis. The primary defect is an inherited mitochondrial genetic abnormality, either from the nuclear genome-coded or mitochondrial genes. The mitochondria in the mutation carriers would be in a delicate balance with the struggle to compensate for such a defect. Compensatory capacity diminishes with ageing and the subsequent accumulation of somatic mitochondrial mutations. Environmental insults, particularly in the presence of genetic susceptibilities to toxicants, would further damage the mitochondria and trigger the decompensation process in motor neurons. Any attempt to compensate via excitatory transmission in the surviving neurons would increase their own metabolic load and adversely affect this delicate balance. These ineffective compensatory attempts then initiate a chain reaction of mitochondrial crisis and neuronal apoptosis, leading to ALS

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