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Variably Protease-Sensitive Prionopathy, a Unique Prion Variant with Inefficient Transmission Properties
Variably protease-sensitive prionopathy (VPSPr) can occur in persons of all codon 129 genotypes in the human prion protein gene (PRNP) and is characterized by a unique biochemical profile when compared with other human prion diseases. We investigated transmission properties of VPSPr by inoculating transgenic mice expressing human PRNP with brain tissue from 2 persons with the valine-homozygous (VV) and 1 with the heterozygous methionine/valine codon 129 genotype. No clinical signs or vacuolar pathology were observed in any inoculated mice. Small deposits of prion protein accumulated in the brains of inoculated mice after challenge with brain material from VV VPSPr patients. Some of these deposits resembled microplaques that occur in the brains of VPSPr patients. Comparison of these transmission properties with those of sporadic Creutzfeldt-Jakob disease in the same lines of mice indicated that VPSPr has distinct biological properties. Moreover, we established that VPSPr has limited potential for human-to-human transmission.
Human prion diseases, also called transmissible spongiform encephalopathies, are a group of rare and inevitably fatal neurodegenerative diseases. Prion diseases are unique in that they occur as idiopathic (sporadic), familial, and acquired disorders. ….
In 2008, a novel prion disease, initially referred to as protease-sensitive prionopathy, was reported in 11 patients who had been referred to the National Prion Disease Pathology Surveillance Center (Cleveland, OH, USA) during May 2002–January 2006. …The defining feature of this group of patients was the unusual biochemical properties of the abnormal PrP in the brain. Compared with the biochemical properties of PrPres in sCJD, the PrPres in VPSPr was found to be much less resistant to protease digestion. ……The neuropathologic features in this group were also unusual, in particular, the accumulation of microplaques within the cerebellum and thalamus, which stained intensely for PrP.
Since the original description of those 11 cases, 19 additional cases have been reported…
….It is possible that the protease-sensitive and the truncated forms of the abnormal PrP may contribute to the unique neuropathology of VPSPr and may also influence the potential for transmission of disease to other persons.
Human-to-human transmission of prion diseases is of great concern for public health reasons (11–13).
……Prion diseases have the potential to be transmissible between persons, a fact that raises public health concerns, particularly regarding vCJD. Assessing the risk for transmission is a challenge because of the varied nature of prion diseases and conflicting evidence over the mechanisms of transmission.
…… We demonstrate that VPSPr is a disease with biological properties distinct from those of sCJD and with a limited, but not negligible, potential for infectivity. These results demonstrate the importance of continuing surveillance to fully uncover the growing spectrum of human prion diseases.
originally posted by: soficrow
Hate to sensationalize, but this is important. A game-changer. World changing. Known for a good long time no doubt, so the important question is, "Why is the truth coming out now?"
First successful vaccination against 'mad cow'-like wasting disease in deer
Researchers at NYU Langone Medical Center and elsewhere say that a vaccination they have developed to fight a brain-based, wasting syndrome among deer and other animals may hold promise on two additional fronts: Protecting U.S. livestock from contracting the disease, and preventing similar brain infections in humans.
originally posted by: lostbook
Wow............Human to human transmission......just when I needed some excitement....NOT
...Have there been any reported cases of human-to-human transmission, or is this just saying that they've now discovered through lab tests that it is a possibility?
Women are at the epicenter of the Alzheimer’s crisis. Almost two-thirds of American seniors living with Alzheimer’s disease are women.
• An estimated 3.2 million women aged 65 and older are living with Alzheimer’s.
• Among those aged 71 and older, 16 percent of women have Alzheimer’s and other dementias, compared with 11 percent of men.
• At age 65, women without Alzheimer’s have more than a one in six chance of developing Alzheimer’s during the remainder of their lives, compared with a one in 11 chance for men.
originally posted by: FalcoFan
a reply to: Silcone Synapse
Thanks-you answered part of the question I had about the effects of this thing on humans.
The other part of the question is-does it make them violent?
Not to over dramatize-but my first thought after reading OP's post was a 28 Days Later scenario.
Dementia and aggressive behaviour
People with dementia may sometimes behave aggressively, either physically or verbally, and this can be very distressing for the person and for their carer, family and friends. This factsheet explains why a person might act aggressively. It gives practical tips that carers can use to help prevent and manage aggressive behaviour. It also looks at the types of support that may be available.
What is aggressive behaviour?
Aggressive behaviour is not unique to people with dementia. However, more than one third of people living with dementia have at times behaved aggressively, particularly in the moderate to severe stages of the condition. ....
Aggressive behaviour may be:
verbal – eg swearing, screaming, shouting, making threats
physical – eg hitting, pinching, scratching, hair-pulling, biting.
originally posted by: damwel
....pardon me if I am not afraid.
originally posted by: damwel
Haven't heard this old stuff since the turn of the century. Mad cow resurfaces, pardon me if I am not afraid.