Staunton, Lisa and Jockusch, Harald and Ohlendieck, Kay
(2011)
Proteomic analysis of muscle affected by motor neuron degeneration:
The wobbler mouse model of amyotrophic lateral sclerosis.
Biochemical and Biophysical Research Communications, 406 (4).
pp. 595-600.
ISSN 0006-291X
Abstract
Amyotrophic lateral sclerosis is the most common form of motor neuron disease in adult patients and
characterized by progressive paralysis. The wobbler mouse (phenotype WR, genotype wr/wr) is an established
animal model of human motor neuron disease and is characterized by a large variety of cellular
abnormalities including muscular atrophy. In analogy to recent proteomic studies of cerebrospinal fluid
and spinal cord, we have used here fluorescence difference in-gel electrophoresis to analyze global
changes in the skeletal muscle proteome from WR versus normal mice. Relative concentrations of 21 proteins
were found to be increased and 3 proteins were decreased. Mass spectrometric analysis identified
these proteins to be associated with key metabolic pathways, the contractile apparatus, intermediate filaments
and the cellular stress response. Drastically increased levels of the glycolytic enzyme glyceraldehyde-
3-phosphate dehydrogenase were confirmed by immunoblotting and this finding agrees with the
idea of an oxidative-to-glycolytic shift in disease-related muscular atrophy. The establishment of novel
disease-specific biomarkers of motor neuron disease might be helpful in the design of improved diagnostic
tools and the identification of novel therapeutic targets.
| Item Type: |
Article
|
| Keywords: |
Amyotrophic lateral sclerosis; DIGE; Motor neuron disease; Muscular atrophy; Muscle proteomics; Wobbler |
| Academic Unit: |
Faculty of Science and Engineering > Biology |
| Item ID: |
7489 |
| Identification Number: |
https://doi.org/10.1016/j.bbrc.2011.02.099 |
| Depositing User: |
Prof. Kay Ohlendieck
|
| Date Deposited: |
07 Oct 2016 10:43 |
| Journal or Publication Title: |
Biochemical and Biophysical Research Communications |
| Publisher: |
Elsevier |
| Refereed: |
Yes |
| Funders: |
Health Research Board (HRB), Fonds der Chemischen Industrie (FCI) |
| URI: |
|
| Use Licence: |
This item is available under a Creative Commons Attribution Non Commercial Share Alike Licence (CC BY-NC-SA). Details of this licence are available
here |
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