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- Title
Dysregulation of axonal transport and motorneuron diseases.
- Authors
Daniela Sau; Paola Rusmini; Valeria Crippa; Elena Bolzoni; Antonia Ratti; Angelo Poletti
- Abstract
MNDs (motorneuron diseases) are neurodegenerative disorders in which motorneurons located in the motor cortex, in the brainstem and in the spinal cord are affected. These diseases in their inherited or sporadic forms are mainly characterized by motor dysfunctions, occasionally associated with cognitive and behavioural alterations. Although these diseases show high variability in onset, progression and clinical symptoms, they share common pathological features, and motorneuronal loss invariably leads to muscle weakness and atrophy. One of the most relevant aspect of these disorders is the occurrence of defects in axonal transport, which have been postulated to be either a direct cause, or a consequence, of motorneuron degeneration. In fact, due to their peculiar morphology and high energetic metabolism, motorneurons deeply rely on efficient axonal transport processes. Dysfunction of axonal transport is known to adversely affect motorneuronal metabolism, inducing progressive degeneration and cell death. In this regard, the understanding of the fine mechanisms at the basis of the axonal transport process and of their possible alterations may help shed light on MND pathological processes. In the present review, we will summarize what is currently known about the alterations of axonal transport found to be either causative or a consequence of MNDs.
- Subjects
AXONAL transport; MOTOR neuron diseases; NEURODEGENERATION; MOTOR cortex; BRAIN stem; SPINAL cord; CELL death; METABOLISM
- Publication
Biology of the Cell (Wiley-Blackwell), 2011, Vol 103, Issue 2, p87
- ISSN
0248-4900
- Publication type
Article
- DOI
10.1042/BC20100093