Featured Research

from universities, journals, and other organizations

Glycosaminoglycans Contribute To Skeletal Abnormalities In Patients With Lysosomal Storage Diseases

Date:
October 22, 2009
Source:
American Journal of Pathology
Summary:
Researchers have demonstrated that glycosaminoglycans (GAGs) contribute to skeletal abnormalities in patients with lysosomal storage diseases.

A group led by Dr. Dieter Brömme at the University of British Columbia has demonstrated that glycosaminoglycans (GAGs) contribute to skeletal abnormalities in patients with lysosomal storage diseases.

Related Articles


Their report can be found in the November 2009 issue of The American Journal of Pathology.

Mucopolysaccharidoses (MPS) are a group of diseases in which the dysfunction of a lysosomal enzyme results in decreased breakdown of GAGs, a type of carbohydrate, in various tissues. These GAGs then collect in cells, causing severe cellular damage that affects bone, skeletal structure, connective tissue, and organs.

In cells that break down bone, GAGs have been shown to inhibit the function of cathepsin K, an enzyme that breaks down collagen, which leads to insufficient space for new bone formation. As MPS patients have severe deficiencies in bone growth and development, Wilson et al hypothesized that cathepsin K inhibition may contribute to bone pathology in MPS patients. They found that both GAGs and cathepsin K were expressed in bone growth regions of a mouse model of MPS type I and that higher levels of cartilage accumulated in bone growth regions of MPS I mice than in their wild-type counterparts. In addition, cathepsin K-mediated collagen degradation was significantly reduced in bone-resorbing cells from MPS I mice.

Taken together, the data by Dr. Brömme and colleagues suggest that "the decrease in the collagenolytic activity of cathepsin K due to the expression of GAGs will greatly reduce osteoclast function and will thus likely contribute to the skeletal abnormalities observed in MPS I bone."


Story Source:

The above story is based on materials provided by American Journal of Pathology. Note: Materials may be edited for content and length.


Journal Reference:

  1. Wilson S, Hashamiyan S, Clarke L, Saftig P, Mort J, Dejica VM, Brömme D. Glycosaminoglycan-Mediated Loss of Cathepsin K Collagenolytic Activity in MPS I Contributes to Osteoclast and Growth Plate Abnormalities. American Journal Of Pathology, 2009; DOI: 10.2353/ajpath.2009.090211

Cite This Page:

American Journal of Pathology. "Glycosaminoglycans Contribute To Skeletal Abnormalities In Patients With Lysosomal Storage Diseases." ScienceDaily. ScienceDaily, 22 October 2009. <www.sciencedaily.com/releases/2009/10/091022071135.htm>.
American Journal of Pathology. (2009, October 22). Glycosaminoglycans Contribute To Skeletal Abnormalities In Patients With Lysosomal Storage Diseases. ScienceDaily. Retrieved October 31, 2014 from www.sciencedaily.com/releases/2009/10/091022071135.htm
American Journal of Pathology. "Glycosaminoglycans Contribute To Skeletal Abnormalities In Patients With Lysosomal Storage Diseases." ScienceDaily. www.sciencedaily.com/releases/2009/10/091022071135.htm (accessed October 31, 2014).

Share This



More Health & Medicine News

Friday, October 31, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Fauci Says Ebola Risk in US "essentially Zero"

Fauci Says Ebola Risk in US "essentially Zero"

AP (Oct. 30, 2014) — NIAID Director Anthony Fauci said the risk of Ebola becoming an epidemic in the U.S. is essentially zero Thursday at the Washington Ideas Forum. He also said an Ebola vaccine will be tested in West Africa in the next few months. (Oct. 30) Video provided by AP
Powered by NewsLook.com
Nurse Defies Ebola Quarantine With Bike Ride

Nurse Defies Ebola Quarantine With Bike Ride

AP (Oct. 30, 2014) — A nurse who vowed to defy Maine's voluntary quarantine for health care workers who treated Ebola patients followed through on her promise Thursday, leaving her home for an hour-long bike ride. (Oct. 30) Video provided by AP
Powered by NewsLook.com
Pot-Infused Edibles Raise Concerns in Colorado

Pot-Infused Edibles Raise Concerns in Colorado

AFP (Oct. 30, 2014) — Colorado may have legalized marijuana for recreational use, but the debate around the decision still continues, with a recent - failed - attempt to ban cannabis-infused edibles. Duration: 01:53 Video provided by AFP
Powered by NewsLook.com
British Navy Ship Arrives in Sierra Leone With Ebola Aid

British Navy Ship Arrives in Sierra Leone With Ebola Aid

AFP (Oct. 30, 2014) — The British ship RFA ARGUS arrived in Sierra Leone to deliver supplies and equipment to help the fight against Ebola. Duration: 00:42 Video provided by AFP
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
 
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:  

Breaking News:

Strange & Offbeat Stories

 

Health & Medicine

Mind & Brain

Living & Well

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:  

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile iPhone Android Web
Follow Facebook Twitter Google+
Subscribe RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins