MNDs (motorneuron diseases) are neurodegenerative disorders in whichmotorneurons located in themotor 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.

Dysregulation of axonal transport and motorneuron diseases / D. Sau, P. Rusmini, V. Crippa, E. Onesto, E. Bolzoni, A. Ratti, A. Poletti. - In: BIOLOGY OF THE CELL. - ISSN 0248-4900. - 103:2(2011 Feb 11), pp. 87-107. [10.1042/BC20100093]

Dysregulation of axonal transport and motorneuron diseases

D. Sau;P. Rusmini;V. Crippa;E. Onesto;E. Bolzoni;A. Ratti;A. Poletti
2011-02-11

Abstract

MNDs (motorneuron diseases) are neurodegenerative disorders in whichmotorneurons located in themotor 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.
Motor neuron ; axonal transport ; SBMA ; ALS ; CMT ; neurodegeneratove diseases
Settore BIO/13 - Biologia Applicata
Settore MED/26 - Neurologia
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/149718
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