Navigation Links
Holy glycosylation! New 'bat signal' flags distressed cells in childhood genetic diseases
Date:6/12/2012

Bethesda, MDJust as Gotham City uses the Bat Signal to call for Batman's aid, a new tool developed by scientists from the Sanford-Burnham Medical Research Institute in La Jolla, California, should serve as the cellular equivalent for children with glycosylation disorders, sometimes called "CDG syndromes." In a new report appearing online in The FASEB Journal (http://www.fasebj.org), scientists describe how they used a green fluorescent protein to identify the presence of genesknown and unknownassociated with a wide variety of glycosylation-related diseases. By being able to identify exactly which genes are defective, researchers can develop treatments and therapies to correct the root causes of these diseases rather than merely treating the symptoms.

Glycosylation is an enzymatic process that coats proteins, lipids or other organic molecules with sugar molecules. It helps cells "stick" together, and proteins fold and work properly, among other things. When this process does not function correctly, it causes diseases involving intellectual disability, digestive problems, seizures and low blood sugar.

"We hope this glowing protein will help light the path for the discovery of new genes that cause genetic disorders in children," said Hudson Freeze, Ph.D., a senior researcher involved in the work from the Genetic Disease Program at Sanford-Burnham Medical Research Institute in La Jolla, California. "It's not Harry Potter's magic wand, but we hope it will offer a way to test for new therapies in these kids. They're counting on us."

To make this advance, Freeze and colleagues engineered cells from children with glycosylation disorders so the cells would glow to indicate when there was a glycosylation problem related to a defective or missing gene. Once the problematic, glowing cells were "rescued" by inserting a healthy gene into the cell or correcting a defective gene's function, the cells stopped glowing. This new tool may be used in high-throughput screening to identify therapeutic molecules that improve glycosylation in defective cells, including stem cells. In addition, this advance may serve as the foundation for a new diagnostic tool for patients.

"These glowing proteins serve as a hotline between distressed cells and researchers hoping to restore their normal function," said Gerald Weissmann, M.D., Editor-in-Chief of The FASEB Journal. "Knowing when and where there are cells with flawed glycosylation pathways should allow researchers to rapidly screen for compounds that may have therapeutic potential."


'/>"/>
Contact: Cody Mooneyhan
cmooneyhan@faseb.org
301-634-7104
Federation of American Societies for Experimental Biology
Source:Eurekalert

Related medicine news :

1. Gatekeeper of brain steroid signals boosts emotional resilience to stress
2. Gene Signal presents data at ARVO 2012 showing topical aganirsen is active in retinal disease
3. Palpitations May Signal Future Heart Rhythm Problem
4. Study Spots Early Warning Signal for Sudden Cardiac Death
5. Re-Routing Spinal Cord Signals Restores Movement in Paralyzed Rats
6. New drug prevents spread of human prostate cancer cells
7. Tiny hitchhikers attack cancer cells
8. To prevent leukemias dreaded return, go for the stem cells
9. Scientists identify major source of cells defense against oxidative stress
10. Therapy exploits addiction of leukemia cells
11. IBN discovers human neural stem cells with tumor targeting ability
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:7/24/2017)... SANTA CLARA, CA (PRWEB) , ... July 24, 2017 , ... ... government and financial organizations, today announced it has partnered with WALLIX to ... NIST Special Publication 800-171 . , There are a number of ways to address ...
(Date:7/24/2017)... ... July 24, 2017 , ... Anyone who uses scales know they ... would a pharmacist using that same scale to dispense medication. The first example is ... These illustrations show why it is important to have the right balance for the ...
(Date:7/24/2017)... ... 24, 2017 , ... A Southern California-based author has released a new book ... she has overcome them. “Forbidden Memories: A Memoir,” by author Michelle V. Madsen, highlights ... risen above. , In “Memories,” readers get a firsthand look at what Michelle has ...
(Date:7/24/2017)... (PRWEB) , ... July 24, 2017 , ... ... how to change manufacturers future. , The agency is hammering out a game ... one of the farthest-ranging laws ever to affect FDA-regulated firms. The new law:, ...
(Date:7/24/2017)... ... 24, 2017 , ... “A Short Walk to the Mailbox”: a remarkable memoir ... of published author, Ed Clark. Ed Clark is a church music director and ... a minister of music and worship leader for over fifty years. He has ...
Breaking Medicine News(10 mins):
(Date:7/10/2017)... S.C. , July 10, 2017  BDI ... purchasing and patient support services organization serving specialty ... today the launch of four significant, value-added member ... market insights, better manage reimbursement and improve access ... and factor therapies. ...
(Date:7/5/2017)... 5, 2017 Oramed Pharmaceuticals Inc. ... ), a clinical-stage pharmaceutical company focused on the development ... received approval from the Israel Securities Authority to dual-list its ... common stock will commence trading on the TASE on July ... market capitalization of the Company, it is expected that Oramed ...
(Date:6/30/2017)... -- AVACEN Medical (AVACEN) announced the publication of new research in the ... its AVACEN Treatment Method to significantly reduce the widespread ... ... ... approximately 200 to 400 million people worldwide according to The National ...
Breaking Medicine Technology: