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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; in revised form: 28 November 2011 / Accepted: 30 November 2011 / Published: 7 December 2011
Abstract: 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.
Keywords: amyotrophic lateral sclerosis; axonal transport; kinesins; dynein; myosin
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Soo, K.Y.; Farg, M.; Atkin, J.D. Molecular Motor Proteins and Amyotrophic Lateral Sclerosis. Int. J. Mol. Sci. 2011, 12, 9057-9082.
Soo KY, Farg M, Atkin JD. Molecular Motor Proteins and Amyotrophic Lateral Sclerosis. International Journal of Molecular Sciences. 2011; 12(12):9057-9082.
Soo, Kai Y; Farg, Manal; Atkin, Julie D. 2011. "Molecular Motor Proteins and Amyotrophic Lateral Sclerosis." Int. J. Mol. Sci. 12, no. 12: 9057-9082.