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

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 dem...

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Published inCellular 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
LanguageEnglish
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
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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.
AbstractList 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.
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.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.
Author Huang, Yu-Ting
Chaytow, Helena
Gillingwater, Thomas H.
Faller, Kiterie M. E.
Author_xml – sequence: 1
  givenname: Helena
  surname: Chaytow
  fullname: Chaytow, Helena
  organization: Euan MacDonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh Medical School: Biomedical Sciences, University of Edinburgh
– sequence: 2
  givenname: Yu-Ting
  surname: Huang
  fullname: Huang, Yu-Ting
  organization: Euan MacDonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh Medical School: Biomedical Sciences, University of Edinburgh
– sequence: 3
  givenname: Thomas H.
  orcidid: 0000-0002-0306-5577
  surname: Gillingwater
  fullname: Gillingwater, Thomas H.
  email: t.gillingwater@ed.ac.uk
  organization: Euan MacDonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh Medical School: Biomedical Sciences, University of Edinburgh
– sequence: 4
  givenname: Kiterie M. E.
  orcidid: 0000-0002-4525-7059
  surname: Faller
  fullname: Faller, Kiterie M. E.
  organization: Euan MacDonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh Medical School: Biomedical Sciences, University of Edinburgh, Royal (Dick) School of Veterinary Studies, University of Edinburgh
BackLink https://www.ncbi.nlm.nih.gov/pubmed/29872871$$D View this record in MEDLINE/PubMed
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Keywords Ubiquitin
Cytoskeleton
Ribonucleoprotein
Translation
Bioenergetic pathway
Spinal muscular atrophy
Language English
License Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
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Snippet Ever since loss of survival motor neuron (SMN) protein was identified as the direct cause of the childhood inherited neurodegenerative disorder spinal muscular...
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SubjectTerms 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
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Title The role of survival motor neuron protein (SMN) in protein homeostasis
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