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.

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 April 18, 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 April 18, 2014).

Share This



More Health & Medicine News

Friday, April 18, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Obama: 8 Million Healthcare Signups

Obama: 8 Million Healthcare Signups

AP (Apr. 17, 2014) — President Barack Obama gave a briefing Thursday announcing 8 million people have signed up under the Affordable Care Act. He blasted continued Republican efforts to repeal the law. (April 17) Video provided by AP
Powered by NewsLook.com
Is Apathy A Sign Of A Shrinking Brain?

Is Apathy A Sign Of A Shrinking Brain?

Newsy (Apr. 17, 2014) — A recent study links apathetic feelings to a smaller brain. Researchers say the results indicate a need for apathy screening for at-risk seniors. Video provided by Newsy
Powered by NewsLook.com
Could Even Casual Marijuana Use Alter Your Brain?

Could Even Casual Marijuana Use Alter Your Brain?

Newsy (Apr. 16, 2014) — A new study conducted by researchers at Northwestern and Harvard suggests even casual marijuana use can alter your brain. Video provided by Newsy
Powered by NewsLook.com
Thousands Of Vials Of SARS Virus Go Missing

Thousands Of Vials Of SARS Virus Go Missing

Newsy (Apr. 16, 2014) — A research institute in Paris somehow misplaced more than 2,000 vials of the deadly SARS virus. Video provided by Newsy
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:
from the past week

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