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Nat Rev Neurosci. 2011 Jan;12(1):31-42. doi: 10.1038/nrn2946.

Hereditary spastic paraplegias: membrane traffic and the motor pathway.

Author information

1
Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 9000 Rockville Pike, Bethesda, Maryland 20892, USA.

Erratum in

  • Nat Rev Neurosci. 2011 Feb;12(2):118.

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.

PMID:
21139634
PMCID:
PMC5584382
DOI:
10.1038/nrn2946
[Indexed for MEDLINE]
Free PMC Article

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