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Prion Test For Rare, Fatal Brain Disease Might Help Diagnose Alzheimer’s, Parkinson’s

Update Date: Feb 07, 2017 08:20 AM EST
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A human prion disease known as classic Creutzfeldt-Jakob Disease (CJD) is a neurodegenerative disease that is rare and fatal. A modified version of the prion test has been shown capable of detecting Alzheimer's and Parkinson's disease.

CJD kills about one million people in the world every year with 300 deaths happening in the United States. Normal proteins in the brain of the affected person start to bend into unnatural shape and even coax other proteins to do the same. The misshapen proteins are known as prions and they form clumps in the brain causing neurons to die.

CJD has no known cause and could just appear with no apparent reason. Other cases are inherited, and a small number have contracted the disease through close contact with an infected person's brain, through transplant or contaminated surgical equipment. Variant CJD is a human version of mad cow disease which is linked to eating infected beef according to Centers for Disease Control and Prevention.

Alzheimer's and Parkinson's disease, also associated with protein misfolding, takes months or years to diagnose according to Byron Caughey, a biochemist at Rocky Mountain Laboratories in Hamilton, Mont., which is a part of the national Institute of Allergy and Infectious Disease.

Recently, Caughey teamed up with scientists Italy, Japan and United Kingdom to develop a test call RT-QuIC which means "real time quaking-induced conversion." It harnesses the bad protein's ability to induce the normal one to take a twisted form.

CJD prions in symptomatic patients have been identified through the test. It is now being distributed to CJD surveillance centers in many countries. Due to its success, Alison Green, a biochemist at the University of Edinburgh in the United Kingdom, is working on a modified version of the test to detect Parkinson's disease and Lewy body dementia.

If the modified prion test will be a reliable diagnostic test for Alzheimer's and Parkinson's disease, doctors could start the patient's therapy as soon as possible. Green explains that drugs and therapies are sometimes ineffective because it is given too late.

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