Creutzfeldt–Jakob disease (CJD), also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is a fatal neurodegenerative disease. Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. Later symptoms include dementia, involuntary movements, blindness, weakness, and coma. About 70% of people die within a year of diagnosis. The name "Creutzfeldt–Jakob disease" was introduced by Walther Spielmeyer in 1922, after the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob.
A prion is a misfolded protein that induces misfolding in normal variants of the same protein, leading to cellular death. Prions are responsible for prion diseases, known as transmissible spongiform encephalopathy (TSEs), which are fatal and transmissible neurodegenerative diseases affecting both humans and animals. These proteins can misfold sporadically, due to genetic mutations, or by exposure to an already misfolded protein, leading to an abnormal three-dimensional structure that can propagate misfolding in other proteins.
The University of California, San Francisco (UCSF), is a public land-grant research university in San Francisco, California, United States. It is part of the University of California system and is dedicated entirely to health science and life science. It conducts research and teaching in medical and biological sciences.
Daniel Carleton Gajdusek was an American physician and medical researcher who was the co-recipient of the Nobel Prize in Physiology or Medicine in 1976 for work on the transmissibility of kuru, implying the existence of an infectious agent, which he named an 'unconventional virus'. In 1996, Gajdusek was charged with child molestation and, after being convicted, spent 12 months in prison before entering a self-imposed exile in Europe, where he died a decade later.
Paul Greengard was an American neuroscientist best known for his work on the molecular and cellular function of neurons. In 2000, Greengard, Arvid Carlsson and Eric Kandel were awarded the Nobel Prize for Physiology or Medicine for their discoveries concerning signal transduction in the nervous system. He was Vincent Astor Professor at Rockefeller University, and served on the Scientific Advisory Board of the Cure Alzheimer's Fund, as well as the Scientific Council of the Brain & Behavior Research Foundation. He was married to artist Ursula von Rydingsvard.
Elizabeth Helen Blackburn is an Australian-American Nobel laureate who is the former president of the Salk Institute for Biological Studies. In 1984, Blackburn co-discovered telomerase, the enzyme that replenishes the telomere, with Carol W. Greider. For this work, she was awarded the 2009 Nobel Prize in Physiology or Medicine, sharing it with Carol W. Greider and Jack W. Szostak, becoming the first Australian woman Nobel laureate.
The virino is a hypothetical infectious particle once theorized to be the cause of scrapie and other degenerative diseases of the central nervous system. It was thought to consist of nucleic acids within a protective coat of host cell proteins. The hypothesis was never widely accepted, and the causative agents responsible for these diseases are now widely accepted to be prions.
Randy Wayne Schekman is an American cell biologist at the University of California, Berkeley, former editor-in-chief of Proceedings of the National Academy of Sciences and former editor of Annual Review of Cell and Developmental Biology. In 2011, he was announced as the editor of eLife, a new high-profile open-access journal published by the Howard Hughes Medical Institute, the Max Planck Society and the Wellcome Trust launching in 2012. He was elected to the National Academy of Sciences in 1992. Schekman shared the 2013 Nobel Prize for Physiology or Medicine with James Rothman and Thomas C. Südhof for their ground-breaking work on cell membrane vesicle trafficking.
The major prion protein (PrP) is encoded in the human body by the PRNP gene also known as CD230. Expression of the protein is most predominant in the nervous system but occurs in many other tissues throughout the body.
Karen K. Hsiao Ashe is a professor at the Department of Neurology and Neuroscience at the University of Minnesota (UMN) Medical School, where she holds the Edmund Wallace and Anne Marie Tulloch Chairs in Neurology and Neuroscience. She is the founding director of the N. Bud Grossman Center for Memory Research and Care, and her specific research interest is memory loss resulting from Alzheimer's disease and related dementias. Her research has included the development of an animal model of Alzheimer's.
Laura Manuelidis is a physician and neuropathologist at Yale University.
In medicine, proteinopathy, or proteopathy, protein conformational disorder, or protein misfolding disease, is a class of diseases in which certain proteins become structurally abnormal, and thereby disrupt the function of cells, tissues and organs of the body.
Kuru is a rare, incurable, and fatal neurodegenerative disorder that was formerly common among the Fore people of Papua New Guinea. Kuru is a form of prion disease which leads to tremors and loss of coordination from neurodegeneration. The term kúru means “trembling” and comes from the Fore word kuria or guria. It is also known as the "laughing sickness" due to the pathologic bursts of laughter which are a symptom of the infection.
The brain, like most other internal organs, or offal, can serve as nourishment. Brains used for nourishment include those of pigs, squirrels, rabbits, horses, cattle, monkeys, chickens, camels, fish, lamb, and goats. In many cultures, different types of brain are considered a delicacy.
Michael J. Homer was an American electronics and computer industry executive who played major roles in the development of the personal computer, mobile devices and the Internet.
Tikvah Alper trained as a physicist and became a distinguished radiobiologist. Among many other initiatives and discoveries, she was among the first to find evidence indicating that the infectious agent in Scrapie does not contain nucleic acid: a finding that was instrumental in understanding the development of the Prion theory. She was director of the MRC Experimental Radiopathology Unit, Hammersmith Hospital, London, UK, 1962–1974.
John Stanley Griffith (1928–1972) was a British chemist, mathematician and biophysicist. He was the nephew of the distinguished British bacteriologist Frederick Griffith.
Konrad Beyreuther is a German molecular biologist and chemist known for his work on neurodegenerative diseases.
Lary Walker is an American neuroscientist and researcher at Emory University in Atlanta, Georgia. He is Associate Director of the Goizueta Alzheimer's Disease Research Center at Emory, and he is known for his research on the role of abnormal proteins in the causation of Alzheimer's disease.
