Type

Journal Article

Authors

Kay Ohlendieck
Martin Clynes
Michael Henry
Dieter Swandulla
Margit Zweyer
Paul Dowling
Ashling Holland

Subjects

Biochemistry

Topics
large scale network structure muscular dystrophy duchenne profiling laminin tissue reveals aged

Proteomic profiling of cardiomyopathic tissue from the aged mdx model of Duchenne muscular dystrophy reveals a drastic decrease in laminin, nidogen and annexin (2013)

Abstract The majority of patients afflicted with Duchenne muscular dystrophy develop cardiomyopathic complications, warranting large-scale proteomic studies of global cardiac changes for the identification of new protein markers of dystrophinopathy. The aged heart from the X-linked dystrophic mdx mouse has been shown to exhibit distinct pathological aspects of cardiomyopathy. In order to establish age-related alterations in the proteome of dystrophin-deficient hearts, cardiomyopathic tissue from young versus aged mdx mice was examined by label-free LC-MS/MS. Significant age-dependent alterations were established for 67 proteins, of which 28 proteins were shown to exhibit a lower abundance and 39 proteins were found to be increased in their expression levels. Drastic changes were demonstrated for 17 proteins, including increases in Ig chains and transferrin, and drastic decreases in laminin, nidogen and annexin. An immunblotting survey of young and old wild-type versus mdx hearts confirmed these proteomic findings and illustrated the effects of natural aging versus dystrophin deficiency. These proteome-wide alterations suggest a disintegration of the basal lamina structure and cytoskeletal network in dystrophin-deficient cardiac fibres, increased levels of antibodies in a potential autoimmune reaction of the degenerating heart, compensatory binding of excess iron and a general perturbation of metabolic pathways in dystrophy-associated cardiomyopathy.
Collections Ireland -> Maynooth University -> Academic Unit = Faculty of Science and Engineering: Biology
Ireland -> Maynooth University -> Type = Article
Ireland -> Maynooth University -> Academic Unit = Faculty of Science and Engineering
Ireland -> Maynooth University -> Status = Published
Ireland -> Maynooth University -> Open Access DRIVERset

Full list of authors on original publication

Kay Ohlendieck, Martin Clynes, Michael Henry, Dieter Swandulla, Margit Zweyer, Paul Dowling, Ashling Holland

Experts in our system

1
Kay Ohlendieck
Maynooth University
Total Publications: 119
 
2
Martin Clynes
Dublin City University
Total Publications: 209
 
3
Michael Henry
Dublin City University
Total Publications: 78
 
4
Dieter Swandulla
Dublin City University
Total Publications: 15
 
5
Margit Zweyer
Dublin City University
Total Publications: 14
 
6
Paul Dowling
Dublin City University
Total Publications: 79
 
7
Ashling Holland
Maynooth University
Total Publications: 9