Diverse role of Survival Motor Neuron Protein.

Diverse role of Survival Motor Neuron Protein.
Biochim Biophys Acta. 2017 Jan 14;:
Authors: Singh RN, Howell MD, Ottesen EW, Singh NN
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.
PMID: 28095296 [PubMed - as supplied by publi...

Source: Biochimica et Biophysica Acta - January 13, 2017 Category: Biochemistry Authors: Singh RN, Howell MD, Ottesen EW, Singh NN Tags: Biochim Biophys Acta Source Type: research