Researchers find mechanism responsible for neuronal death in prion diseases

Prion diseases, such as mad cow disease in cattle and Creutzfeldt-Jakob disease (CJD) in humans--have stumped scientists for decades with a complex "whodunnit" complete with many suspects and a missing murder weapon.

The brain of a victim killed by prion diseases is clogged with clumps of prion protein (PrP) in a rare, misfolded state called PrPSc. Controversy has long raged about how these diseases get started and if the clumps of PrPSc actually kill brain cells--in some forms of the disease clumps aren't even there. The culprit responsible for the death of neurons is still a mystery.

Now research from Whitehead Institute Director Susan Lindquist and Jiyan Ma, now at Ohio State University, suggests a unifying theory that can help explain how these devastating diseases get started and how they kill. The results, published in two papers in the October 17, 2002 online issue of the journal Science, show that small amounts of PrP accumulating in the cellular space called the cytosol kill neurons in cultured cells and transgenic mice. Mice suffered from neurodegeneration and loss of muscle control, similar to patients with CJD and other prion diseases.

PrP normally sits on the cell surface. But if it misfolds before it gets to the surface, the cell's quality control mechanism sends it to the cytosol for destruction. The new research shows that if the quality control system becomes overwhelmed, even a small amount of PrP in the cytosol will kill the neuron.

In an accompanying paper, the lab reports also that when misfolded PrP accumulates in the cytosol, it can sometimes convert to the clumpy PrPSc-like form. A key feature of prion hypothesis is the "infectious" nature of its shape change--once PrPSc forms, it can coax other prion proteins into this alternative folding pattern and cause them to clump together. Lindquist and Ma have found a mechanism by which this infectious shape change can get started and demonstrated its self-propaga

Contact: Nadia Halim
Whitehead Institute for Biomedical Research

Page: 1 2 3 4 5

Related biology news :

1. Researchers determine genetic cause of Timothy syndrome
2. Researchers find color sensitive atomic switch in bacteria
3. Researchers identify protein promoting vascular tumor growth
4. Researchers devise potent new tools to curb ivory poaching
5. Researchers create nanotubes that change colors, form nanocarpet and kill bacteria
6. Researchers ID chlorophyll-regulating gene
7. Researchers develop fast track way to discover how cells are regulated
8. Researchers identify distinctive signature for metastatic prostate cancer
9. Researchers report new gene test for isolated cleft lip and palate
10. Researchers discover why mutant gene causes colon cancer
11. Researchers identify the genomes controlling elements

Post Your Comments:

(Date:8/3/2020)... ... August 03, 2020 , ... Accumen Inc. ... with medical equipment manufacturer, Spectrum Solutions, to provide COVID-19 saliva testing kits nationally. ... supply chain for more than 10 years. Early in the COVID-19 crisis, we ...
(Date:7/18/2020)... ... July 17, 2020 , ... Commercial launch readiness is a critical ... discover a COVID cure or vaccine, the global economic downturn will only increase price ... not going away and capturing full value from every product launch is critical. However, ...
(Date:7/2/2020)... (PRWEB) , ... July 02, ... ... Biology Systems, B.V. (MBS) has announced a publication detailing the use of ... reverse transcription polymerase chain reaction (RT-PCR) in 16 minutes. The article, titled ...
Breaking Biology News(10 mins):
(Date:7/18/2020)... ... July 17, 2020 , ... dicentra , a leading ... food industries, is pleased to announce that Charles Galea has joined its clinical ... Charles is an accomplished and results-driven sales executive with over 10 years of ...
(Date:7/10/2020)... , ... July 09, 2020 ... ... company, announced today that Massachusetts Institute of Technology (MIT) has expanded the ... license allows PathSensors to move into the point-of-care diagnostic market, focusing initially ...
(Date:7/10/2020)... ... July 09, 2020 , ... ... today announced the hiring of Allen R. Nissenson, M.D., F.A.C.P., as its Chief ... clinical development of Sentien’s lead product, SBI-101. Dr. Nissenson serves as an ...
(Date:7/7/2020)... ... , ... Bio-IT World has announced the winners of the ... of Chicago, Massachusetts General Hospital, Mission: Cure, and the Pistoia Alliance were honored. ... examples of how technology innovations and strategic initiatives can be powerful forces for ...
Breaking Biology Technology:
Cached News: