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:3/30/2017)... 30, 2017  On April 6-7, 2017, Sequencing.com will ... hackathon at Microsoft,s headquarters in ... focus on developing health and wellness apps that provide ... the Genome is the first hackathon for personal ... largest companies in the genomics, tech and health industries ...
(Date:3/28/2017)... , March 28, 2017 ... Biometrics), Hardware (Camera, Monitors, Servers, Storage Devices), Software (Video ... and Region - Global Forecast to 2022", published by ... in 2016 and is projected to reach USD 75.64 ... 2017 and 2022. The base year considered for the ...
(Date:3/23/2017)... March 23, 2017 The report "Gesture Recognition and Touchless ... and Geography - Global Forecast to 2022", published by MarketsandMarkets, the market is ... of 29.63% between 2017 and 2022. Continue Reading ... ... ...
Breaking Biology News(10 mins):
(Date:10/11/2017)... ... ... wash is a basic first aid supply for any work environment, but most personal eye ... first if a dangerous substance enters both eyes? It’s one less decision, and likely quicker ... eye piece. , “Whether its dirt and debris, or an acid or alkali, getting anything ...
(Date:10/11/2017)... ... , ... Disappearing forests and increased emissions are the main causes of the ... Especially those living in larger cities are affected by air pollution related diseases. , ... pollution-affected countries globally - decided to take action. , “I knew I had to ...
(Date:10/10/2017)... (PRWEB) , ... October 10, 2017 , ... ... Science Center’s FirstHand program has won a US2020 STEM Mentoring Award. Representatives of ... award for Excellence in Volunteer Experience from US2020. , US2020’s mission is to ...
(Date:10/10/2017)... PITTSBURGH, PA (PRWEB) , ... October 10, 2017 ... ... this year’s recipients of 13 prestigious awards honoring scientists who ... be presented in a scheduled symposium during Pittcon 2018, the world’s leading conference ...
Breaking Biology Technology:
Cached News: