Molecular motor proteins and amyotrophic lateral sclerosis

Kai Y. Soo, Manal Farg, Julie D. Atkin

Research output: Contribution to journalReview articleResearchpeer-review

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.

LanguageEnglish
Pages9057-9082
Number of pages26
JournalInternational Journal of Molecular Sciences
Volume12
Issue number12
DOIs
Publication statusPublished - Dec 2011
Externally publishedYes

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Molecular Motor Proteins
Dyneins
Amyotrophic Lateral Sclerosis
Neurons
Motor Neurons
neurons
proteins
Proteins
Kinesin
Axonal Transport
paralysis
Defects
Paralysis
Neurodegenerative Diseases
axons
sheds
Transgenic Mice
Brain Stem
spinal cord
Pathology

Cite this

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Molecular motor proteins and amyotrophic lateral sclerosis. / Soo, Kai Y.; Farg, Manal; Atkin, Julie D.

In: International Journal of Molecular Sciences, Vol. 12, No. 12, 12.2011, p. 9057-9082.

Research output: Contribution to journalReview articleResearchpeer-review

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