Next Article in Journal
Investigating Ionic Effects Applied to Water Based Organocatalysed Aldol Reactions
Next Article in Special Issue
Differential Motor Neuron Impairment and Axonal Regeneration in Sporadic and Familiar Amyotrophic Lateral Sclerosis with SOD-1 Mutations: Lessons from Neurophysiology
Previous Article in Journal
Enhanced Immunomodulatory Activity of Gelatin-Encapsulated Rubus coreanus Miquel Nanoparticles
Previous Article in Special Issue
Crossing the Border: Molecular Control of Motor Axon Exit
Article Menu

Export Article

Open AccessReview
Int. J. Mol. Sci. 2011, 12(12), 9057-9082;

Molecular Motor Proteins and Amyotrophic Lateral Sclerosis

Department of Biochemistry, La Trobe Institute of Molecular Science, La Trobe University, VIC 3086, Australia
Centre for Neuroscience, University of Melbourne, Parkville, VIC 3010, Australia
Florey Neuroscience Institute, University of Melbourne, Parkville, VIC 3010, Australia
Author to whom correspondence should be addressed.
Received: 11 October 2011 / Revised: 28 November 2011 / Accepted: 30 November 2011 / Published: 7 December 2011
(This article belongs to the Special Issue Studies of Motor Molecules)
Full-Text   |   PDF [228 KB, uploaded 19 June 2014]


Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder affecting motor neurons in the brain, brainstem and spinal cord, which is characterized by motor dysfunction, muscle dystrophy and progressive paralysis. Both inherited and sporadic forms of ALS share common pathological features, however, the initial trigger of neurodegeneration remains unknown. Motor neurons are uniquely targeted by ubiquitously expressed proteins in ALS but the reason for this selectively vulnerability is unclear. However motor neurons have unique characteristics such as very long axons, large cell bodies and high energetic metabolism, therefore placing high demands on cellular transport processes. Defects in cellular trafficking are now widely reported in ALS, including dysfunction to the molecular motors dynein and kinesin. Abnormalities to dynein in particular are linked to ALS, and defects in dynein-mediated axonal transport processes have been reported as one of the earliest pathologies in transgenic SOD1 mice. Furthermore, dynein is very highly expressed in neurons and neurons are particularly sensitive to dynein dysfunction. Hence, unravelling cellular transport processes mediated by molecular motor proteins may help shed light on motor neuron loss in ALS. View Full-Text
Keywords: amyotrophic lateral sclerosis; axonal transport; kinesins; dynein; myosin amyotrophic lateral sclerosis; axonal transport; kinesins; dynein; myosin
This is an open access article distributed under the Creative Commons Attribution License (CC BY 3.0).

Share & Cite This Article

MDPI and ACS Style

Soo, K.Y.; Farg, M.; Atkin, J.D. Molecular Motor Proteins and Amyotrophic Lateral Sclerosis. Int. J. Mol. Sci. 2011, 12, 9057-9082.

Show more citation formats Show less citations formats

Related Articles

Article Metrics

Article Access Statistics



[Return to top]
Int. J. Mol. Sci. EISSN 1422-0067 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top