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The role of survival motor neuron protein (SMN) in protein homeostasis.
- Source :
-
Cellular and molecular life sciences : CMLS [Cell Mol Life Sci] 2018 Nov; Vol. 75 (21), pp. 3877-3894. Date of Electronic Publication: 2018 Jun 05. - Publication Year :
- 2018
-
Abstract
- 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.
- Subjects :
- Animals
Autophagy
Cytoskeleton genetics
Cytoskeleton metabolism
Cytoskeleton pathology
Endocytosis
Energy Metabolism
Humans
Muscular Atrophy, Spinal genetics
Muscular Atrophy, Spinal pathology
Protein Biosynthesis
Ribonucleoproteins genetics
Ribonucleoproteins metabolism
SMN Complex Proteins genetics
Ubiquitin genetics
Ubiquitin metabolism
Muscular Atrophy, Spinal metabolism
Proteostasis
SMN Complex Proteins metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 1420-9071
- Volume :
- 75
- Issue :
- 21
- Database :
- MEDLINE
- Journal :
- Cellular and molecular life sciences : CMLS
- Publication Type :
- Academic Journal
- Accession number :
- 29872871
- Full Text :
- https://doi.org/10.1007/s00018-018-2849-1