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Article
Diverse role of survival motor neuron protein
Biochimica et Biophysica Acta (BBA)— Gene Regulatory Mechanisms
  • Ravindra N. Singh, Iowa State University
  • Matthew D. Howell, Iowa State University
  • Eric W. Ottesen, Iowa State University
  • Natalia N. Singh, Iowa State University
Document Type
Article
Publication Version
Accepted Manuscript
Publication Date
3-1-2017
DOI
10.1016/j.bbagrm.2016.12.008
Abstract

The multifunctional Survival Motor Neuron (SMN) protein is required for the survival of all organisms of the animal kingdom. SMN impacts various aspects of RNA metabolism through the formation and/or interaction with ribonucleoprotein (RNP) complexes. SMN regulates biogenesis of small nuclear RNPs, small nucleolar RNPs, small Cajal body-associated RNPs, signal recognition particles and telomerase. SMN also plays an important role in DNA repair, transcription, pre-mRNA splicing, histone mRNA processing, translation, selenoprotein synthesis, macromolecular trafficking, stress granule formation, cell signaling and cytoskeleton maintenance. The tissue-specific requirement of SMN is dictated by the variety and the abundance of its interacting partners. Reduced expression of SMN causes spinal muscular atrophy (SMA), a leading genetic cause of infant mortality. SMA displays a broad spectrum ranging from embryonic lethality to an adult onset. Aberrant expression and/or localization of SMN has also been associated with male infertility, inclusion body myositis, amyotrophic lateral sclerosis and osteoarthritis. This review provides a summary of various SMN functions with implications to a better understanding of SMA and other pathological conditions.

Comments

This is a manuscript of an article published as Singh, Ravindra N., Matthew D. Howell, Eric W. Ottesen, and Natalia N. Singh. "Diverse role of survival motor neuron protein." Biochimica et Biophysica Acta (BBA)—Gene Regulatory Mechanisms 1860, no. 3 (2017): 299-315. DOI: 10.1016/j.bbagrm.2016.12.008. Posted with permission.

Creative Commons License
Creative Commons Attribution-Noncommercial-No Derivative Works 4.0
Copyright Owner
Elsevier B.V.
Language
en
File Format
application/pdf
Citation Information
Ravindra N. Singh, Matthew D. Howell, Eric W. Ottesen and Natalia N. Singh. "Diverse role of survival motor neuron protein" Biochimica et Biophysica Acta (BBA)— Gene Regulatory Mechanisms Vol. 1860 Iss. 3 (2017) p. 299 - 315
Available at: http://works.bepress.com/ravindra-singh/34/