Home  |  Top News  |  Most Popular  |  Video  |  Multimedia  |  News Feeds  |  Feedback
  Medicine  |  Nature & Earth  |  Biology  |  Technology & Engineering  |  Space & Planetary  |  Psychology  |  Physics & Chemistry  |  Economics  |  Archaeology
Top > Medicine, Health Care > New Vitamin-based Treatment That Could… >

New Vitamin-based Treatment That Could Reduce Muscle Degeneration in Muscular Dystrophy

Published: October 23, 2012.
Released by Public Library of Science  

Boosting the activity of a vitamin-sensitive cell adhesion pathway has the potential to counteract the muscle degeneration and reduced mobility caused by muscular dystrophies, according to a research team led by scientists at the University of Maine.

The discovery, published 23 October in the open access journal PLOS Biology, is particularly important for congenital muscular dystrophies, which are progressive, debilitating and often lethal diseases that currently remain without cure. The researchers found that they could improve muscle structure and function in a zebrafish version of muscular dystrophy by supplying a common cellular chemical (or its precursor, vitamin B3) to activate a cell adhesion pathway.

Muscle cells are in themselves relatively delicate, but derive important additional mechanical strength from adhesion protein complexes; these anchor the muscle cells to an external framework known as the basement membrane, thereby helping to buffer the cells against the extreme forces that they experience during muscle contractions. Mutations in the genes that encode these adhesion proteins can weaken these attachments, making muscle cells more susceptible to damage and death.

The resulting muscle degeneration can eventually lead to progressive muscle-wasting diseases, such as muscular dystrophies. A major component of the basement membrane, a protein called laminin, binds to multiple different receptors on the muscle cell surface and forms a dense, organized network.

The study was led by UMaine Associate Professor of Biological Sciences, Clarissa Henry, whose laboratory focuses on understanding how cell adhesion complexes contribute to muscle development. The researchers discovered that a pathway involving a common cellular chemical called nicotinamide adenine dinucleotide (NAD+) plays a role in the formation of organized basement membranes in muscle tissue, during development of the fish embryo. As disordered basement membranes are seen in many different types of muscular dystrophies, the researchers wondered whether activating this pathway might reduce the severity of some muscular dystrophies.

In the current study, the researchers show that NAD+ improves the organization of laminin in a zebrafish version of muscular dystrophy. Zebrafish lacking either of the two main receptors for laminin have a disorganized basement membrane, causing muscle degeneration and difficulties with movement. However adding extra NAD+, or even a vitamin packet containing vitamin B3 (niacin, a precursor to NAD+), significantly reduced these symptoms.

The research team found that the main protective effects of NAD+ come from enhancing the organization of the laminin structure in the basement membrane, which helps to increase the resilience of diseased muscle fibers.

Because the same cell adhesion complexes are found in humans, the research team is optimistic that these findings may one day positively impact patients with muscular dystrophies. "Although there is a long way to go, I'm hopeful that our data could eventually lead to new adjuvant therapies," says University of Maine Ph.D. student Michelle Goody, who led the research team with Prof. Henry.

Prof. Henry summarizes; "One of my favorite aspects of this study is that it is a poster child for how asking basic biological questions can lead to exciting discoveries that may have future therapeutic potential."




The above story is based on materials provided by Public Library of Science.

Translate this page: Chinese French German Italian Japanese Korean Portuguese Russian Spanish


comments powered by Disqus


Related »

Stem 
12/17/14 
Stem Cells Faulty in Duchenne Muscular Dystrophy, Stanford Researchers Find
Like human patients, mice with a form of Duchenne muscular dystrophy undergo progressive muscle degeneration and accumulate connective tissue …
Dystrophin 
4/1/13 
Promoting Muscle Regeneration in a Mouse Model of Muscular Dystrophy
Duchenne muscular dystrophy (DMD) is a degenerative skeletal muscle disease caused by mutations in the protein dystrophin. Dystrophin functions …
Muscular 
1/15/15 
Hope for Muscular Dystrophy Patients: Harnessing Gene Helps Repair Muscle Damage
Researchers have successfully improved the ability of muscle to repair itself - by artificially increasing levels of the BMI1 …
Muscle 
1/22/13 

Scientists Discover 'Needle in a Haystack' for Muscular Dystrophy Patients
Dystrophin 
12/7/12 

Another Muscular Dystrophy Mystery Solved; MU Scientists Inch Closer to a Therapy for Patients
Mkp-5 
4/1/13 
JCI Early Table of Contents for April 1, 2013
Promoting muscle regeneration in a mouse model of muscular dystrophy …
Muscular 
11/27/12 
Protein Injection Points to Muscular Dystrophy Treatment
November 27, 2012 — Ottawa — Scientists have discovered that injecting a novel human protein into muscle affected by …
Stem 
6/1/12 
Potential New Approach to Regenerating Skeletal Muscle Tissue
An innovative strategy for regenerating skeletal muscle tissue using cells derived from the amniotic fluid is outlined in new …
Fibrosis 
10/14/14 
Out-of-step Cells Spur Muscle Fibrosis in Duchenne Muscular Dystrophy Patients
Like a marching band falling out of step, muscle cells fail to perform in unison in patients with Duchenne …
More » 
 
© Newsline Group  |  About  |  Privacy Policy  |  Feedback  |  Mobile