Oxidative Stress-Induced Axon Fragmentation Is a Consequence of Reduced Axonal Transport in Hereditary Spastic Paraplegia SPAST Patient Neurons

Abstract excerpt

Hereditary Spastic Paraplegia (HSP) is a group of inherited disorders characterised by progressive spasticity and paralysis of the lower limbs. Autosomal dominant mutations in SPAST gene account for ~40% of adult-onset patients. We have previously shown that SPAST patient cells have reduced organelle transport and are therefore more sensitive to oxidative stress. To test whether these effects are present in...

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