Type

Journal Article

Authors

Kay Ohlendieck
Dieter Swandulla
Rustam R Mundegar
Margit Zweyer
Michael Henry
Paula Meleady
Paul Dowling
Ashling Holland

Subjects

Biochemistry

Topics
tissue damage extracellular matrix proteins protein binding cytoskeletal proteins muscular dystrophy duchenne body fluids animal model factor analysis

Label-free mass spectrometric analysis of the mdx-4cv diaphragm identifies the matricellular protein periostin as a potential factor involved in dystrophinopathy-related fibrosis. (2014)

Abstract Proteomic profiling plays a decisive role in the identification of novel biomarkers of muscular dystrophy and the elucidation of new pathobiochemical mechanisms that underlie progressive muscle wasting. Building on the findings of recent comparative analyses of tissue samples and body fluids from dystrophic animals and patients afflicted with Duchenne muscular dystrophy, we have used here label-free MS to study the severely dystrophic diaphragm from the not extensively characterized mdx-4cv mouse. This animal model of progressive muscle wasting exhibits less dystrophin-positive revertant fibers than the conventional mdx mouse, making it ideal for the future monitoring of experimental therapies. The pathoproteomic signature of the mdx-4cv diaphragm included a significant increase in the fibrosis marker collagen and related extracellular matrix proteins (asporin, decorin, dermatopontin, prolargin) and cytoskeletal proteins (desmin, filamin, obscurin, plectin, spectrin, tubulin, vimentin, vinculin), as well as decreases in proteins of ion homeostasis (parvalbumin) and the contractile apparatus (myosin-binding protein). Importantly, one of the most substantially increased proteins was identified as periostin, a matricellular component and apparent marker of fibrosis and tissue damage. Immunoblotting confirmed a considerable increase of periostin in the dystrophin-deficient diaphragm from both mdx and mdx-4cv mice, suggesting an involvement of this matricellular protein in dystrophinopathy-related fibrosis.
Collections Ireland -> Dublin City University -> PubMed

Full list of authors on original publication

Kay Ohlendieck, Dieter Swandulla, Rustam R Mundegar, Margit Zweyer, Michael Henry, Paula Meleady, Paul Dowling, Ashling Holland

Experts in our system

1
Kay Ohlendieck
Maynooth University
Total Publications: 131
 
2
Dieter Swandulla
Maynooth University
Total Publications: 26
 
3
Rustam R Mundegar
Maynooth University
Total Publications: 17
 
4
Margit Zweyer
Maynooth University
Total Publications: 24
 
5
Michael Henry
Dublin City University
Total Publications: 99
 
6
Paula Meleady
Dublin City University
Total Publications: 115
 
7
Paul Dowling
Maynooth University
Total Publications: 82