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Prion Diseases (Transmissible Spongiform Encephalopathies)

A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA; ATAXIA; and a fatal outcome. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The older literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83)
Also Known As:
Transmissible Spongiform Encephalopathies; Transmissible Spongiform Encephalopathy; Prion Disease; Inherited Human Transmissible Spongiform Encephalopathies; Transmissible Dementias; Encephalopathies, Spongiform, Transmissible; Human Transmissible Spongiform Encephalopathies, Inherited; Prion Protein Diseases; Prion-Induced Disorder; Dementia, Transmissible; Disorder, Prion-Induced; Disorders, Prion-Induced; Encephalopathies, Transmissible Spongiform; Encephalopathy, Transmissible Spongiform; Prion Induced Disorder; Prion Protein Disease; Prion-Induced Disorders; Spongiform Encephalopathy, Transmissible; Transmissible Dementia; Dementias, Transmissible; Spongiform Encephalopathies, Transmissible
Networked: 2133 relevant articles (44 outcomes, 152 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Alzheimer Disease (Alzheimer's Disease)
2. Neurodegenerative Diseases (Neurodegenerative Disease)
3. Parkinson Disease (Parkinson's Disease)
4. Scrapie
5. Infection

Experts

1. Collinge, John: 57 articles (10/2015 - 01/2003)
2. Aguzzi, Adriano: 29 articles (12/2015 - 06/2002)
3. Soto, Claudio: 27 articles (05/2015 - 08/2002)
4. Gambetti, Pierluigi: 26 articles (10/2015 - 12/2002)
5. Dong, Xiao-Ping: 25 articles (12/2015 - 08/2005)
6. Brown, David R: 25 articles (09/2015 - 06/2002)
7. Caughey, Byron: 25 articles (01/2015 - 03/2002)
8. Prusiner, Stanley B: 22 articles (10/2015 - 03/2003)
9. Carp, Richard I: 21 articles (01/2015 - 11/2002)
10. Shi, Qi: 20 articles (12/2015 - 09/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Prion Diseases:
1. AntibodiesIBA
2. Quinacrine (Mepacrine)IBA
3. PrionsIBA
4. Amyloid (Amyloid Fibrils)IBA
5. Proteins (Proteins, Gene)IBA
6. CopperIBA
7. RNA (Ribonucleic Acid)IBA
8. PrPSc ProteinsIBA
9. Monoclonal AntibodiesIBA
10. Protein Isoforms (Isoforms)IBA

Therapies and Procedures

1. Blood Transfusion (Blood Transfusions)
2. Therapeutics
3. Immunotherapy
4. Surgical Instruments (Clip)
5. Transplants (Transplant)