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:3/15/2016)... March 15, 2016 --> ... by Transparency Market Research "Digital Door Lock Systems Market - ... - 2023," the global digital door lock systems market in ... 2014 and is forecast to grow at a CAGR of ... small and medium enterprises (MSMEs) across the world and high ...
(Date:3/11/2016)... http://www.apimages.com ) - --> http://www.apimages.com ... at AP Images ( http://www.apimages.com ) - Germany ... produce the new refugee identity cards. DERMALOG will be unveiling this ... Hanover next week.   --> ... to produce the new refugee identity cards. DERMALOG will be unveiling ...
(Date:3/9/2016)... YORK , March 9, 2016 This ... and future states of the RNA Sequencing (RNA Seq) ... segments such as instruments, tools and reagents, data analysis, ... Analyze various segments of the RNA-Sequencing market such as ... services Identify the main factors affecting each segment and ...
Breaking Biology News(10 mins):
(Date:5/3/2016)... ... May 03, 2016 , ... Morf ... and compliance training platform on mobile devices, today released a new interactive ... for Medical Devices. The course is essential for owners or operators of places ...
(Date:5/3/2016)... ... 2016 , ... Kerafast Inc., developers of an online ... globe, today announced the availability of a Zika virus antibody from Vanderbilt University. ... prevention measures for the Zika virus, the virus’s geographical distribution continues to widen ...
(Date:5/3/2016)... New York, NY (PRWEB) , ... May 03, 2016 , ... ... Lenox Hill Hospital , for definitive prostate cancer treatment, patients traditionally had two main ... an appropriate treatment plan would be made. , New technology has enabled doctors ...
(Date:5/2/2016)... ... May 02, 2016 , ... StarNet Communications Corp, ( http://www.starnet.com/ ) a leading ... Secure Remote Desktop modules to its flagship X-Win32 PC X server. The new ... to the user’s PC over encrypted SSH. , Traditionally, users of PC X servers ...
Breaking Biology Technology: