Abstract
| Original language | English |
|---|---|
| Pages (from-to) | 4546-4557 |
| Number of pages | 12 |
| Journal | Journal of Proteome Research |
| Volume | 13 |
| Issue number | 11 |
| Early online date | 29 Aug 2014 |
| DOIs | |
| Publication status | Published - 2014 |
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Keywords
- proteomic profiling
- spinal muscular atrophy
- motor neurons
Cite this
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Label-free quantitative proteomic profiling identifies disruption of ubiquitin homeostasis as a key driver of schwann cell defects in spinal muscular atrophy. / Aghamaleky Sarvestany, Arwin; Hunter, Gillian; Tavendale, Amy; Lamont, Douglas; Llavero Hurtado, Maica; Graham, Laura; Wishart, Thomas; Gillingwater, Thomas.
In: Journal of Proteome Research, Vol. 13, No. 11, 2014, p. 4546-4557.Research output: Contribution to journal › Article
TY - JOUR
T1 - Label-free quantitative proteomic profiling identifies disruption of ubiquitin homeostasis as a key driver of schwann cell defects in spinal muscular atrophy
AU - Aghamaleky Sarvestany, Arwin
AU - Hunter, Gillian
AU - Tavendale, Amy
AU - Lamont, Douglas
AU - Llavero Hurtado, Maica
AU - Graham, Laura
AU - Wishart, Thomas
AU - Gillingwater, Thomas
N1 - Publisher version not permitted in repositories, set to back end vis. ET 30-11-15
PY - 2014
Y1 - 2014
N2 - Low levels of survival of motor neuron (SMN) protein cause the neuromuscular disease spinal muscular atrophy (SMA), characterized by degeneration of lower motor neurons and atrophy of skeletal muscle. Recent work demonstrated that low levels of SMN also trigger pathological changes in Schwann cells, leading to abnormal axon myelination and disrupted deposition of extracellular matrix proteins in peripheral nerve. However, the molecular pathways linking SMN depletion to intrinsic defects in Schwann cells remained unclear. Label-free proteomics analysis of Schwann cells isolated from SMA mouse peripheral nerve revealed widespread changes to the Schwann cell proteome, including disruption to growth/proliferation, cell death/survival, and molecular transport pathways. Functional clustering analyses revealed significant disruption to a number of proteins contributing to ubiquitination pathways, including reduced levels of ubiquitin-like modifier activating enzyme 1 (Uba1).
AB - Low levels of survival of motor neuron (SMN) protein cause the neuromuscular disease spinal muscular atrophy (SMA), characterized by degeneration of lower motor neurons and atrophy of skeletal muscle. Recent work demonstrated that low levels of SMN also trigger pathological changes in Schwann cells, leading to abnormal axon myelination and disrupted deposition of extracellular matrix proteins in peripheral nerve. However, the molecular pathways linking SMN depletion to intrinsic defects in Schwann cells remained unclear. Label-free proteomics analysis of Schwann cells isolated from SMA mouse peripheral nerve revealed widespread changes to the Schwann cell proteome, including disruption to growth/proliferation, cell death/survival, and molecular transport pathways. Functional clustering analyses revealed significant disruption to a number of proteins contributing to ubiquitination pathways, including reduced levels of ubiquitin-like modifier activating enzyme 1 (Uba1).
KW - proteomic profiling
KW - spinal muscular atrophy
KW - motor neurons
U2 - 10.1021/pr500492j
DO - 10.1021/pr500492j
M3 - Article
VL - 13
SP - 4546
EP - 4557
JO - Journal of Proteome Research
JF - Journal of Proteome Research
SN - 1535-3893
IS - 11
ER -