Hereditary spastic paraplegias: membrane traffic and the motor pathway

C Blackstone, CJ O'kane, E Reid - Nature Reviews Neuroscience, 2011 - nature.com
Nature Reviews Neuroscience, 2011nature.com
Voluntary movement is a fundamental way in which animals respond to, and interact with,
their environment. In mammals, the main CNS pathway controlling voluntary movement is
the corticospinal tract, which encompasses connections between the cerebral motor cortex
and the spinal cord. Hereditary spastic paraplegias (HSPs) are a group of genetic disorders
that lead to a length-dependent, distal axonopathy of fibres of the corticospinal tract, causing
lower limb spasticity and weakness. Recent work aimed at elucidating the molecular cell …
Abstract
Voluntary movement is a fundamental way in which animals respond to, and interact with, their environment. In mammals, the main CNS pathway controlling voluntary movement is the corticospinal tract, which encompasses connections between the cerebral motor cortex and the spinal cord. Hereditary spastic paraplegias (HSPs) are a group of genetic disorders that lead to a length-dependent, distal axonopathy of fibres of the corticospinal tract, causing lower limb spasticity and weakness. Recent work aimed at elucidating the molecular cell biology underlying the HSPs has revealed the importance of basic cellular processes — especially membrane trafficking and organelle morphogenesis and distribution — in axonal maintenance and degeneration.
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