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.