Variant CJD -- Prion amplification breakthrough brings new insights and hopes for a blood test

July 04, 2007

Researchers have shown that a recently developed laboratory method to amplify prions (Protein Misfolding Cyclic Amplification) can be applied to variant CJD (Creutzfeldt-Jakob Disease). The work was carried out by scientists at the National CJD Surveillance Unit at the University of Edinburgh, the Scottish National Blood Transfusion Service, Neuropathogenesis Unit and CSL Behring. It is published this month in the Journal of Pathology.

The team, led by Dr. Mark Head, also shows for the first time that variant CJD prions can be amplified from brain tissue samples using normal blood cells to improve the sensitivity of current detection tests. This method has the potential to be applied on other tissues and fluids, including blood. The prion amplification is dependent on genetic factors, similar to those influencing susceptibility to variant CJD.

Background

Creutzfeldt-Jakob disease (CJD) seems to result from conversion of a normal protein in the body to an abnormal form that is self-replicating as a prion and toxic to the brain. In variant CJD, this occurs after infection by the bovine spongiform encephalopathy (BSE) prion. Following exposure to BSE, there is a long silent period before the prion spreads to the brain and causes neurological symptoms. It is now clear that during this silent period individuals can pass variant CJD prions on to others by blood transfusion and there are also fears that the disease might also be spread by certain kinds of surgery.

One way to protect blood recipients from this threat is to screen blood donations for prions, but efforts to develop such a test have proven difficult, partly because of the very low level of prions that are likely to be present in blood.

The team stress that the work is at an early stage, but co-researcher Professor James Ironside, of the National CJD Surveillance Unit at the University of Edinburgh, said "These new findings provide us with an invaluable tool to study one of the fundamental aspects of variant CJD and take us one step closer towards supporting a test to screen for individuals who might inadvertently pass this disease on to others through blood transfusion, organ donation or surgery."
-end-


Wiley

Related Brain Articles from Brightsurf:

Glioblastoma nanomedicine crosses into brain in mice, eradicates recurring brain cancer
A new synthetic protein nanoparticle capable of slipping past the nearly impermeable blood-brain barrier in mice could deliver cancer-killing drugs directly to malignant brain tumors, new research from the University of Michigan shows.

Children with asymptomatic brain bleeds as newborns show normal brain development at age 2
A study by UNC researchers finds that neurodevelopmental scores and gray matter volumes at age two years did not differ between children who had MRI-confirmed asymptomatic subdural hemorrhages when they were neonates, compared to children with no history of subdural hemorrhage.

New model of human brain 'conversations' could inform research on brain disease, cognition
A team of Indiana University neuroscientists has built a new model of human brain networks that sheds light on how the brain functions.

Human brain size gene triggers bigger brain in monkeys
Dresden and Japanese researchers show that a human-specific gene causes a larger neocortex in the common marmoset, a non-human primate.

Unique insight into development of the human brain: Model of the early embryonic brain
Stem cell researchers from the University of Copenhagen have designed a model of an early embryonic brain.

An optical brain-to-brain interface supports information exchange for locomotion control
Chinese researchers established an optical BtBI that supports rapid information transmission for precise locomotion control, thus providing a proof-of-principle demonstration of fast BtBI for real-time behavioral control.

Transplanting human nerve cells into a mouse brain reveals how they wire into brain circuits
A team of researchers led by Pierre Vanderhaeghen and Vincent Bonin (VIB-KU Leuven, Université libre de Bruxelles and NERF) showed how human nerve cells can develop at their own pace, and form highly precise connections with the surrounding mouse brain cells.

Brain scans reveal how the human brain compensates when one hemisphere is removed
Researchers studying six adults who had one of their brain hemispheres removed during childhood to reduce epileptic seizures found that the remaining half of the brain formed unusually strong connections between different functional brain networks, which potentially help the body to function as if the brain were intact.

Alcohol byproduct contributes to brain chemistry changes in specific brain regions
Study of mouse models provides clear implications for new targets to treat alcohol use disorder and fetal alcohol syndrome.

Scientists predict the areas of the brain to stimulate transitions between different brain states
Using a computer model of the brain, Gustavo Deco, director of the Center for Brain and Cognition, and Josephine Cruzat, a member of his team, together with a group of international collaborators, have developed an innovative method published in Proceedings of the National Academy of Sciences on Sept.

Read More: Brain News and Brain Current Events
Brightsurf.com is a participant in the Amazon Services LLC Associates Program, an affiliate advertising program designed to provide a means for sites to earn advertising fees by advertising and linking to Amazon.com.