HOME >> BIOLOGY >> NEWS
Disruption of protein-folding causes neurodegeneration, mental retardation

Excess accumulation in brain cells of a fat molecule called GM1-ganglioside (GM1) disrupts the folding of newly assembled proteins into their proper shapes, triggering nerve degeneration and mental retardation in children. This finding, from investigators at St. Jude Children's Research Hospital, is published in the Sept. 10 issue of Molecular Cell.

The disease, called GM1 gangliosidosis, disrupts the normal function of brain cells and causes them to self-destruct. The St. Jude discovery offers strong evidence for the cause of GM1 gangliosidosis in children. GM1 gangliosidosis is a lysosomal storage disorders, an inherited disease in which one or more enzymes in the lysosomes are defective. Lysosomes are the cell's recycling centers, where proteins, fats and other molecules are broken down into their basic building blocks, which are then reused to make new molecules.

Lysosomal storage diseases occur when lysosomes lack the enzymes they need to perform their recycling tasks, leading to abnormal accumulation of the molecules the lysosome is supposed to break down. These diseases are responsible for most severe cases of nerve degeneration and mental retardation among children.

The discovery identifies for the first time the endoplasmic reticulum (ER), the cell's protein processing factory as the location of biochemical reactions leading to brain cell death in children with this disease.

"Our finding is exciting because children with GM1 gangliosidosis are severely affected and their outlooks are dismal," said Alessandra d'Azzo, Ph.D., member of the St. Jude department of Genetics and Tumor Cell Biology. "Now that we have a better understanding of what causes the damage, we may be able to design treatments that specifically remedy this problem. If this finding holds true for other lysosomal storage diseases, the impact could be especially fruitful."

d'Azzo is senior author of the Molecular Cell report.

GM1, a type of fatty molecule ca
'"/>

Contact: Bonnie Cameron
bonnie.cameron@stjude.org
901-495-4815
St. Jude Children's Research Hospital
9-Sep-2004


Page: 1 2 3

Related biology news :

1. Studies Document Ease Of Ecosystem Disruption
2. Killer Whales Have Begun Preying On Sea Otters, Causing Disruption Of Coastal Ecosystems In Western Alaska
3. Social Disruption Leads To Shorter Survival In Monkeys With AIDS
4. Cigarette smoke causes breaks in DNA and defects to a cells chromosomes, Pitt study finds
5. Pollutant causes delayed flowering in plants
6. Researchers discover why mutant gene causes colon cancer
7. Analysis fingers causes of desertification
8. Vioxx in high doses causes hypertension
9. Gene mutation causes progressive changes to cell structure in children with Progeria
10. Late invasion of infected cells into the brain causes HIV dementia say Temple researchers
11. Fat cell hormone causes weight loss

Post Your Comments:
*Name:
*Comment:
*Email:


(Date:2/2/2019)... ... January 31, 2019 , ... ... geared toward clinical operations executives and focusing on all facets of clinical trial ... project management, portfolio management, resource management, patient engagement, and more. , “We ...
(Date:1/30/2019)... ... January 30, 2019 , ... ... supports high-growth, high-impact life science and biotechnology companies, announced today that it ... foundations and disease-focused venture funds to discuss their investment philosophies, explain their ...
(Date:1/24/2019)... ... 2019 , ... rHEALTH LLC, an innovative digital health solutions ... United States Patent and Trademark Office (USPTO) for U.S. Patent Application No. 14/682,010, ... application covers the addition of a length scale to hydrogel microparticles. A ...
Breaking Biology News(10 mins):
(Date:1/30/2019)... , ... January 30, 2019 , ... ... from pharma, academia and governmental agencies to discuss best practices for laboratory automation ... February 4-6. , At this year’s conference, Visikol will be at booth ...
(Date:1/24/2019)... ... January 22, 2019 , ... Controlled Contamination Services is ... in the US. Each of these valuable new team members will support its growing ... model moving forward as CCS scales its business. This addition will enable CCS to ...
(Date:1/21/2019)... FRANKLIN, Wis. (PRWEB) , ... January 21, 2019 ... ... company, announced their newest product line, SporActiv™, for the industrial and institutional cleaning ... a variety of residential and commercial cleaning needs. Applications include odor control, floor ...
(Date:1/20/2019)... ... January 17, 2019 , ... Today, Advanced Solutions, Inc., a ... specialized automation technology, titled Automate 3D. Automate 3D, produced by Advanced Solutions, allows ... to deliver to end-users, customers, and ERP systems. , Automate 3D ...
Breaking Biology Technology:
Cached News: