Navigation Links
Sarcospan, a little protein for a big problem
Date:11/2/2008

The overlooked and undervalued protein, sarcospan, just got its moment in the spotlight. Peter et al. now show that adding it to muscle cells might ameliorate the most severe form of muscular dystrophy.

In Duchenne muscular dystrophy (DMD), the mutated dystrophin protein fails to anchor correctly to its membrane glycoprotein complex. And without this anchoring, muscle cells experience severe contraction-induced damage. Sarcospan is part of the anchoring complex, but because mice without sarcospan don't seem any worse for its absence, it hasn't received much attention. Sarcospan's structure, however, suggests it might help stabilize the membrane complex, so the authors decided to test the effects of increasing sarcospan expression in a DMD mouse model.

The increase did not improve the dystrophinglycoprotein interaction, but instead, the team was surprised to find sarcospan coaxed a dystrophin relative called utrophin to spread out on the muscle membrane. Utrophin is normally restricted to the neuromuscular junction, where it serves a role similar to that of dystrophin.

The extra sarcospan prompted higher levels of utrophin in the cell, but not by increasing its expression. Sarcospan instead stabilized extrajunctional utrophin complexes, which normally form early in development and then disappear after the first few weeks of life.

Mouse muscle cells were protected by sarcospan, but the true importance of this discovery will lie in its potential for human therapeutics, specifically gene therapy. In that regard, sarcospan's small gene size is significantat 600 bp, it is easily packaged into the safest viral vectors, unlike either dystrophin or utrophin, which are about 700 times larger and require more immunogenic vectors.


'/>"/>

Contact: Sati Motieram
motierd@mail.rockefeller.edu
212-327-8581
Rockefeller University Press
Source:Eurekalert

Page: 1

Related biology news :

1. Beetles get by with a little help from their friends
2. Modest CO2 cutbacks may be too little, too late for coral reefs
3. A little nitrogen can go a long way
4. Building a stronger roof over your head: 3 little pigs project begins first tests
5. Little teeth suggest big jump in primate timeline
6. MSU researcher uses grant to study little-known but largely useful microbes
7. Getting better with a little help from our micro friends
8. Too much or too little weight gain poses risks to pregnant mothers, babies
9. Variety is the spice of life: too many males, too little time...
10. Ancient lemurs little finger poses mystery
11. Book on little-known species and conservation provides guidance to managers and others
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:12/20/2016)... , Dec. 20, 2016 The ... sharing, rental and leasing is stoking significant interest ... radio frequency technology, Bluetooth low energy (BLE), biometrics ... as the next wave of wireless technologies in ... access system to advanced access systems opens the ...
(Date:12/16/2016)... , Dec. 16, 2016 The global wearable medical ... 12.14 billion by 2021 from USD 5.31 billion in 2016, at ... ... mainly driven by technological advancements in medical devices, launch of a ... preference for wireless connectivity among healthcare providers, and increasing focus on ...
(Date:12/15/2016)... Mich. , Dec. 15, 2016  There is ... car doors or starting the engine. Continental will demonstrate ... Las Vegas . Through the combination ... Start and Entry) and biometric elements, the international technology ... of vehicle personalization and authentication. "The integration ...
Breaking Biology News(10 mins):
(Date:1/11/2017)... (PRWEB) , ... January 11, 2017 , ... ... international society for optics and photonics , are commending the U.S. Congress and ... the signing Friday by the President of the American Innovation and Competitiveness Act ...
(Date:1/11/2017)... Ca (PRWEB) , ... January 11, 2017 , ... ... in pathogens are observed in clinical settings, it is becoming increasingly clear that ... An over-reliance on culture-based methods, the standard in the study of clinical resistance, ...
(Date:1/11/2017)... PA (PRWEB) , ... January 11, 2017 , ... ... entrepreneur can make all the difference when navigating the challenges young businesses face. ... tap into the extensive expertise and experience of Geoff DiMasi, Founder and Principal ...
(Date:1/11/2017)... ... January 11, 2017 , ... Advanced Polymer Monitoring Technologies (APMT) ... Bartylla will lead European initiatives for APMT’s product lines serving polymer and biopharmaceutical ... to European manufacturers and researchers. Bernhard brings significant experience in our application areas ...
Breaking Biology Technology: