HOME >> BIOLOGY >> NEWS
Protein is key to fatal disorder and normal cell function

DURHAM, N.C. The aberrant protein that causes juvenile Batten disease, a fatal neurodegenerative disorder of childhood, also plays a key role in normal cell function, Duke University Medical Center researchers have found.

The Duke study is the first to identify where the cln3 protein resides in human brain cells and to link the protein's location to its function. The researchers discovered that cln3 transports a vital lipid, or complex fat molecule, within a cell. The breakdown of this transportation system results in uncontrolled apoptosis, or cell death, and the neural degeneration that is a hallmark of Batten disease.

"Genetic diseases such as Batten disease provide a unique opportunity to understand the role of proteins, like cln3, that are vital for normal cell function," said Rose-Mary Boustany, M.D., a professor of pediatrics and neurobiology at Duke University Medical Center and senior author of the study.

The results appear in the September 2004 issue of Pediatric Research. The study was supported by the National Institute of Neurological Disorders and Stroke, a division of the National Institutes of Health.

Understanding the role of cln3 has implications beyond juvenile Batten disease a rare, untreatable disorder -- because the protein is overproduced by cells in a number of cancers. And the lipid transported by cln3 plays an important role in infection by the AIDS virus and prion proteins, and in developing Alzheimer's disease. Prions are infectious proteins that can transmit diseases such as Creutzfeld-Jacob or mad cow disease.

Batten disease collectively refers to inherited disorders also called the neuronal ceroid lipofuscinoses, or NCL, which share similar clinical and pathological traits but are genetically distinct. All NCL disorders cause progressive loss of motor skills, mental retardation, loss of speech, blindness, uncontrolled seizures and, eventually, premature death.

The juvenile form of Batte
'"/>

Contact: Becky Oskin
Becky.oskin@duke.edu
919-684-4148
Duke University Medical Center
15-Sep-2004


Page: 1 2 3

Related biology news :

1. Protein is key for digestive function of the pancreas
2. Proteins show promise for mosquito control
3. Protein involved in childhood disorder linked to cancer
4. Protein fishing in America: The movie
5. Protein vaccine fully protects mice from lethal aerosol challenge with ricin toxin
6. Protein key to trafficking in nerve terminals
7. Protein controls acid in cells by direct detection of volume changes, study finds
8. Protein believed to control formation of memory identified by Scripps & UCSD scientists
9. Protein stops blood-vessel growth, holds promise as cancer therapy
10. Proteins transform DNA into molecular velcro
11. Protein engineered to detect nerve gas

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


(Date:8/26/2020)... ... 2020 , ... Modality Solutions, a biopharmaceutical cold chain validation engineering ... The list is the most prestigious ranking of the nation’s fastest-growing private companies. Modality ... list, reported three-year revenue growth of 71 percent. , The Inc. 5000 list ...
(Date:8/21/2020)... ... August 19, 2020 , ... ... and SalivaBio LLC, businesses dedicated to Salivary Bioscience for more than twenty years. ... published book by Springer, " Salivary Bioscience: Foundations of Interdisciplinary Saliva Research and ...
(Date:8/12/2020)... ... 2020 , ... Both Roche, based in Basel, Switzerland, and ... with Housey Pharma’s HMI subsidiary to gain access to its core-enabling technology for ... Development spending in excess of US $10 billion. , Scientists at Housey Pharma ...
Breaking Biology News(10 mins):
(Date:7/31/2020)... ... July 29, 2020 , ... eSource has long been touted as ... cover the history of eSource, the reasons it did not take off as quickly ... site source, the industry is moving towards capturing data electronically for clinical trials and ...
(Date:7/22/2020)... TORONTO (PRWEB) , ... July 22, 2020 , ... Join ... and John Lorenc, Sr. Manager Regulatory Solutions, in a one hour live webinar ... is the regulating body in China for drugs and medical devices. Specifically, for medical ...
(Date:7/18/2020)... Ohio (PRWEB) , ... July 16, 2020 , ... ... data solutions to aid in the early detection and prevention of high-burden diseases, ... to enable US-based hospitals and healthcare organizations to utilize existing data in order ...
(Date:7/10/2020)... ... July 09, 2020 , ... ... today that Massachusetts Institute of Technology (MIT) has expanded the company’s exclusive ... PathSensors to move into the point-of-care diagnostic market, focusing initially on the ...
Breaking Biology Technology:
Cached News: