In 1997, UCSF neurologist Stanley Prusiner won the Nobel Prize for his discovery of infectious proteins called “prions” that cause mad cow disease. That revelation has led to an increased understanding of neurodegenerative diseases such as Alzheimer’s, Parkinson’s and ALS. We talk with Stanley Prusiner about the future of brain disease research and his new book, “Madness and Memory: The Discovery of Prions — A New Biological Principle of Disease.”

UCSF Neurologist’s Mad Cow Discovery Improves Understanding of Degenerative Brain Diseases 22 April,2014forum

Stanley Prusiner, director of the Institute for Neurodegenerative Diseases and professor of neurology at UCSF; and author of "Madness and Memory: The Discovery of Prions -- A New Biological Principle of Disease"

  • Guest

    What is the status of research into the consumption of traces of metals as a cause of brain diseases like Alzheimers? For instance, nuts contain copper, and water filtration devices that use KDF55 filters add copper to their output. Is copper intake linked to any brain disease?

  • Me2b_me

    Am I part of your research, I have right to know? I am sick but no one seems to care

  • Me2b_me

    What if metals were intentionally put into your body to cause these diseases? It seems to me that all these scientist are getting awarded and prized for the things that I have accomplished for them. When they done using you they toss you away right?

  • Debbie Neff McKee

    What is the difference between the old variant CJD and and new variant CJD (mad cow)? Are the prions different? My mother in law died of the old variant per the autopsy, Just wondering how this determination was made (she vacationed in England during the infectious period for Mad Cow.)

    • east_bay_sailor

      In my mother’s case they told me the new variant usually had a different signature in the MRI scans, she also was diagnosed with sporadic CJD even though she was in the UK during the infectious period. We also had the genetic test done, it was a relief that she did not have the inheritable form!

  • east_bay_sailor

    In 2002 and 2003 my mother was treated for CJD by Dr. Prusiner’s colleague Dr. Geschwind, I really appreciate the care she received before she passed away. At the time they were experimenting with quinacrine, I met one patient who had been taking it for about a year and seemed to be maintaining. I’d like to know what the results of that study are (will have to listen to podcast later)…

  • Chemist150

    Misfolding proteins are a common cause of illness. An example with ALS is that a group was took a small segment that displayed dementia associated with ALS and from only 7 patients (small population) they were able to identify 5 unreported proline residues. (Han-Xiang Deng, et al. NATURE. 477(7363), 211-215 (2011))

    The thought is that the cyclic nature of the proline was preventing proper folding of proteins.

    Thus, a real cure is not going to happen. However, if you can suppress the expression/replication of the misfolded protein and stimulate the proper protein, one might, in theory, be able to minimize the disease.

  • Bruce

    Is there a need to study brains of deceased dementia/Alzheimer’s patients? My mother has severe dementia diagnosed as Alzheimer’s and is currently in a nursing home. As her designated health care surrogate and Attorney in Fact, I’m interested in offering her brain to qualified researchers for study upon her death. Are there such programs and how may I contact reputable programs?

    • bb

      Definitely. Very sorry about your mom. I worked in a nursing home when I was 18 and saw many people with this disease. Contact the ADDF – Alzheimer’s Drug Discovery Foundation – would be a good place to start to put you in touch with a researcher nearby.

  • Liz

    I was diagnosed with PD 18 months ago and seem to be helped by taking azilect. What criteria is used for putting patients on dopamine?

    • east_bay_sailor

      Sorry, what is PD?

    • east_bay_sailor

      Never mind, I looked it up, PD = Parkinson’s disease. I don’t mean to ask dumb questions, I was thinking mostly about Dr. Prusiner’s work on CJD.

  • Thomas

    Is there any research on positive uses of prions, such as pest control or antibiotics?

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