Hereditary spastic paraplegia (HSP) includes a large and diverse group of genetic disorders whose main feature is progressive spasticity and weakness in the lower limbs, as a result of continuous distal axonopathy caused by defects in the mechanisms that transport proteins and substances along the axons [90, 91].
Fink, "Hereditary spastic paraplegia: clinico-pathologic features and emerging molecular mechanisms," Acta Neuropathologica, vol.
FSP is also called
hereditary spastic paraplegia and StrA1/4mpell-Lorrain syndrome1.
The importance of er in axons is suggested by the fact that mutations of er-shaping proteins result in
hereditary spastic paraplegia (hsp), A motor axon degeneration disease.
Many mutations for the motor axon degeneration disease,
hereditary spastic paraplegia (HSP), affect proteins that model ER shape.
Objective:
Hereditary spastic paraplegias are a group of rare neurodegenerative diseases due to the degeneration of the long axons of cortical motor neurons.
