Document Detail


Spinal Muscular Atrophy and the Antiapoptotic Role of Survival of Motor Neuron (SMN) Protein.
MedLine Citation:
PMID:  23315303     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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.
Authors:
Ryan S Anderton; Bruno P Meloni; Frank L Mastaglia; Sherif Boulos
Related Documents :
24855953 - Npas4 regulates excitatory-inhibitory balance within neural circuits through cell-type-...
24752543 - Comparing axonal excitability in past polio to amyotrophic lateral sclerosis.
3801923 - The projection to the mesencephalon from the sensory trigeminal nuclei. an anatomical s...
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-1-13
Journal Detail:
Title:  Molecular neurobiology     Volume:  -     ISSN:  1559-1182     ISO Abbreviation:  Mol. Neurobiol.     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-1-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8900963     Medline TA:  Mol Neurobiol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Centre for Neuromuscular and Neurological Disorders, University of Western Australia and Australian Neuromuscular Research Institute, Nedlands, Western Australia, Australia, 20112159@student.uwa.edu.au.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Key elements in adverse drug interaction safety signals: an assessment of individual case safety rep...
Next Document:  Current Issues in GLP-1 Receptor Agonist Therapy for Type 2 Diabetes.