Dowling, Paul and Culligan, Kevin and Ohlendieck, Kay
(2002)
Distal mdx muscle groups exhibiting up-regulation of utrophin
and rescue of dystrophin-associated glycoproteins exemplify
a protected phenotype in muscular dystrophy.
Naturwissenschaften, 89 (2).
pp. 75-78.
ISSN 0028-1042
Abstract
Unique unaffected skeletal muscle fibres, unlike
necrotic torso and limb muscles, may pave the way
for a more detailed understanding of the molecular pathogenesis
of inherited neuromuscular disorders and help to
develop new treatment strategies for muscular dystrophies.
The sparing of extraocular muscle in Duchenne
muscular dystrophy is mostly attributed to the special
protective properties of extremely fast-twitching smalldiameter
fibres, but here we show that distal muscles also
represent a particular phenotype that is more resistant to
necrosis. Immunoblot analysis of membranes isolated
from the well established dystrophic animal model mdx
shows that, in contrast to dystrophic limb muscles, the toe
musculature exhibits an up-regulation of the autosomal
dystrophin homologue utrophin and a concomitant rescue
of dystrophin-associated glycoproteins. Thus distal mdx
muscle groups provide a cellular system that naturally
avoids myofibre degeneration which might be useful in
the search for naturally occurring compensatory mechanisms
in inherited skeletal muscle diseases.
| Item Type: |
Article
|
| Keywords: |
Distal mdx; muscle groups; up-regulation; utrophin; dystrophin-associated glycoproteins; protected phenotype; muscular dystrophy; |
| Academic Unit: |
Faculty of Science and Engineering > Biology |
| Item ID: |
7341 |
| Identification Number: |
https://doi.org/10.1007/s00114-001-0289-4 |
| Depositing User: |
Paul Dowling
|
| Date Deposited: |
15 Aug 2016 16:05 |
| Journal or Publication Title: |
Naturwissenschaften |
| Publisher: |
Springer |
| Refereed: |
Yes |
| Funders: |
Health Research Board (HRB), Enterprise Ireland (EI), Royal Society, London, Royal Irish Academy |
| 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 |
Repository Staff Only(login required)
 |
Item control page |
Downloads per month over past year
Origin of downloads
Altmetric
Altmetric