Carberry, Steven and Zweyer, Margit and Swandulla, Dieter and Ohlendieck, Kay
(2012)
Proteomics reveals drastic increase of extracellular matrix
proteins collagen and dermatopontin in the aged mdx
diaphragm model of Duchenne muscular dystrophy.
International Journal of Molecular Medicine, 30 (2).
pp. 229-234.
ISSN 1107-3756
Abstract
Duchenne muscular dystrophy is a lethal genetic
disease of childhood caused by primary abnormalities in the gene
coding for the membrane cytoskeletal protein dystrophin. The
mdx mouse is an established animal model of various aspects
of X-linked muscular dystrophy and is widely used for studying
fundamental mechanisms of dystrophinopathy and testing
novel therapeutic approaches to treat one of the most frequent
gender-specific diseases in humans. In order to determine global
changes in the muscle proteome with the progressive deterioration
of mdx tissue with age, we have characterized diaphragm
muscle from mdx mice at three ages (8-weeks, 12-months and
22-months) using mass spectrometry-based proteomics. Altered
expression levels in diaphragm of 8-week vs. 22-month mice
were shown to occur in 11 muscle-associated proteins. Aging in
the mdx diaphragm seems to be associated with a drastic increase
in the extracellular matrix proteins, collagen and dermatopontin,
the molecular chaperone αB-crystallin, and the intermediate
filament protein vimentin, suggesting increased accumulation
of connective tissue, an enhanced cellular stress response
and compensatory stabilization of the weakened membrane
cytoskeleton. These proteomic findings establish the aged mdx
diaphragm as an excellent model system for studying secondary
effects of dystrophin deficiency in skeletal muscle tissue.
Item Type: |
Article
|
Additional Information: |
This is an open-access article licensed under a Creative Commons Attribution-NonCommercial 3.0 Unported License. The article may be redistributed, reproduced, and reused for non-commercial purposes, provided the original source is properly cited: Carberry, S., Zweyer, M., Swandulla, D., Ohlendieck, K."Proteomics reveals drastic increase of extracellular matrix proteins collagen and dermatopontin in the aged mdx diaphragm model of Duchenne muscular dystrophy". International Journal of Molecular Medicine 30, no. 2 (2012): 229-234. http://dx.doi.org/10.3892/ijmm.2012.1006 |
Keywords: |
collagen; dermatopontin; diaphragm; muscular dystrophy;
proteomics; |
Academic Unit: |
Faculty of Science and Engineering > Biology |
Item ID: |
6902 |
Identification Number: |
https://doi.org/10.3892/ijmm.2012.1006 |
Depositing User: |
Prof. Kay Ohlendieck
|
Date Deposited: |
21 Jan 2016 14:41 |
Journal or Publication Title: |
International Journal of Molecular Medicine |
Publisher: |
Spandidos Publications |
Refereed: |
Yes |
Funders: |
Muscular Dystrophy Ireland, Duchenne Ireland, Hume scholarship, Health Research Board (HRB), Higher Education Authority (HEA) |
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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