The role of survival motor neuron protein (SMN) in protein homeostasis

• Published in: Cellular and molecular life sciences : CMLS Vol. 75; no. 21; pp. 3877 - 3894
• Main Authors: Chaytow, Helena, Huang, Yu-Ting, Gillingwater, Thomas H., Faller, Kiterie M. E.
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01.11.2018; Springer Nature B.V
• Subjects: Animals; Atrophy; Autophagy; Biochemistry; Bioenergetics; biogenesis; Biomedical and Life Sciences; Biomedicine; Biosynthesis; Cell Biology; Cell survival; childhood; Children; Cytoskeleton; Cytoskeleton - genetics; Cytoskeleton - metabolism; Cytoskeleton - pathology; Endocytosis; Energy Metabolism; Homeostasis; Humans; Life Sciences; Localization; messenger RNA; Mitochondria; Molecular chains; motor neurons; mRNA; muscular atrophy; Muscular Atrophy, Spinal - genetics; Muscular Atrophy, Spinal - metabolism; Muscular Atrophy, Spinal - pathology; Mutation; Neurodegenerative diseases; Neuromuscular diseases; Neurons; Phagocytosis; Proteasomes; Protein Biosynthesis; Proteins; Proteostasis; Review; Ribonucleoproteins; Ribonucleoproteins - genetics; Ribonucleoproteins - metabolism; SMN Complex Proteins - genetics; SMN Complex Proteins - metabolism; SMN protein; Spinal cord; Spinal muscular atrophy; spliceosomes; Survival; translation (genetics); Ubiquitin; Ubiquitin - genetics; Ubiquitin - metabolism; Ubiquitin; Cytoskeleton; Ribonucleoprotein; Translation; Bioenergetic pathway; Spinal muscular atrophy
• ISSN: 1420-682X; 1420-9071; 1420-9071
• DOI: 10.1007/s00018-018-2849-1

Ever since loss of survival motor neuron (SMN) protein was identified as the direct cause of the childhood inherited neurodegenerative disorder spinal muscular atrophy, significant efforts have been made to reveal the molecular functions of this ubiquitously expressed protein. Resulting research demonstrated that SMN plays important roles in multiple fundamental cellular homeostatic pathways, including a well-characterised role in the assembly of the spliceosome and biogenesis of ribonucleoproteins. More recent studies have shown that SMN is also involved in other housekeeping processes, including mRNA trafficking and local translation, cytoskeletal dynamics, endocytosis and autophagy. Moreover, SMN has been shown to influence mitochondria and bioenergetic pathways as well as regulate function of the ubiquitin–proteasome system. In this review, we summarise these diverse functions of SMN, confirming its key role in maintenance of the homeostatic environment of the cell.