A prion is a misfolded protein that can induce misfolding of normal variants of the same protein and trigger cellular death. Prions cause prion diseases known as transmissible spongiform encephalopathies (TSEs) that are transmissible, fatal neurodegenerative diseases in humans and animals. The proteins may misfold sporadically, due to genetic mutations, or by exposure to an already misfolded protein. The consequent abnormal three-dimensional structure confers on them the ability to cause misfolding of other proteins.
The University of California, San Francisco (UCSF) is a public land-grant research university in San Francisco, California. 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.
Scrapie is a fatal, degenerative disease affecting the nervous systems of sheep and goats. It is one of several transmissible spongiform encephalopathies (TSEs), and as such it is thought to be caused by a prion. Scrapie has been known since at least 1732 and does not appear to be transmissible to humans. However, it has been found to be experimentally transmissible to humanised transgenic mice and non-human primates.
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'.
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.
Transmissible mink encephalopathy (TME) is a rare sporadic disease that affects the central nervous system of ranch-raised adult mink. It is a transmissible spongiform encephalopathy, caused by proteins called prions.
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.
Sir John Anthony Hardy is a human geneticist and molecular biologist at the Reta Lila Weston Institute of Neurological Studies at University College London with research interests in neurological diseases.
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. Often the proteins fail to fold into their normal configuration; in this misfolded state, the proteins can become toxic in some way or they can lose their normal function. The proteinopathies include such diseases as Creutzfeldt–Jakob disease and other prion diseases, Alzheimer's disease, Parkinson's disease, amyloidosis, multiple system atrophy, and a wide range of other disorders. The term proteopathy was first proposed in 2000 by Lary Walker and Harry LeVine.
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 transmissible spongiform encephalopathy (TSE) caused by the transmission of abnormally folded proteins (prions), which leads to symptoms such as tremors and loss of coordination from neurodegeneration.
Richard Lewis Huganir is a Bloomberg Distinguished Professor in the Departments of Neuroscience and Psychological and Brain Sciences, Director of the Solomon H. Snyder Department of Neuroscience, and co-director of the Johns Hopkins Medicine Brain Science Institute at the Johns Hopkins University School of Medicine. He has joint appointments in the Department of Biological Chemistry and the Department of Pharmacology and Molecular Sciences in the Johns Hopkins School of Medicine.
Gladstone Institutes is an independent, non-profit biomedical research organization whose focus is to better understand, prevent, treat and cure cardiovascular, viral and neurological conditions such as heart failure, HIV/AIDS and Alzheimer's disease. Its researchers study these diseases using techniques of basic and translational science. Another focus at Gladstone is building on the development of induced pluripotent stem cell technology by one of its investigators, 2012 Nobel Laureate Shinya Yamanaka, to improve drug discovery, personalized medicine and tissue regeneration.
Thomas Christian Südhof, ForMemRS, is a German-American biochemist known for his study of synaptic transmission. Currently, he is a professor in the school of medicine in the department of molecular and cellular physiology, and by courtesy in neurology, and in psychiatry and behavioral sciences at Stanford University.
Gregor Karl Wenning was a German neurologist best known for his clinical and scientific work in Parkinson's disease and atypical Parkinsonian disorders, particularly multiple system atrophy (MSA). In 2006 he was appointed Professor and Head of the Division of Clinical Neurobiology at the Medical University Innsbruck. Wenning died on 11 February 2024, at the age of 59.
Kristine Yaffe is an American Cognitive decline and dementia researcher. She is the Scola Endowed Chair and Vice Chair and Professor of Psychiatry, Neurology and Epidemiology and the Director of the Center for Population Brain Health at the University of California, San Francisco. In 2019, Yaffe was elected to the National Academy of Medicine.
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.
