Journal article
Spinal muscular atrophy and the Antiapoptotic role of Survival of Motor Neuron (SMN) protein
Molecular Neurobiology, Vol.47(2), pp.821-832
2013
Abstract
Spinal muscular atrophy (SMA) is a devastating and often fatal neurodegenerative disease that affects spinal motor neurons and leads to progressive muscle wasting and paralysis. The survival of motor neuron (SMN) gene is mutated or deleted in most forms of SMA, which results in a critical reduction in SMN protein. Motor neurons appear particularly vulnerable to reduced SMN protein levels. Therefore, understanding the functional role of SMN in protecting motor neurons from degeneration is an essential prerequisite for the design of effective therapies for SMA. To this end, there is increasing evidence indicating a key regulatory antiapoptotic role for the SMN protein that is important in motor neuron survival. The aim of this review is to highlight key findings that support an antiapoptotic role for SMN in modulating cell survival and raise possibilities for new therapeutic approaches.
Details
- Title
- Spinal muscular atrophy and the Antiapoptotic role of Survival of Motor Neuron (SMN) protein
- Authors/Creators
- R.S. Anderton (Author/Creator) - The University of Western AustraliaB.P. Meloni (Author/Creator) - The University of Western AustraliaF.L. Mastaglia (Author/Creator) - The University of Western AustraliaS. Boulos (Author/Creator) - The University of Western Australia
- Publication Details
- Molecular Neurobiology, Vol.47(2), pp.821-832
- Publisher
- Humana Press Inc
- Identifiers
- 991005545038607891
- Murdoch Affiliation
- Murdoch University
- Language
- English
- Resource Type
- Journal article
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- Collaboration types
- Domestic collaboration
- Citation topics
- 1 Clinical & Life Sciences
- 1.255 Musculoskeletal Disorders
- 1.255.2204 Spinal Muscular Atrophy
- Web Of Science research areas
- Neurosciences
- ESI research areas
- Neuroscience & Behavior