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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-Associated Disorders; Prion-Induced Disorder; Prion-Induced Disorders; Dementia, Transmissible; Disorder, Prion-Induced; Disorders, Prion-Induced; Encephalopathies, Transmissible Spongiform; Encephalopathy, Transmissible Spongiform; Prion Induced Disorder; Prion Protein Disease; Spongiform Encephalopathy, Transmissible; Transmissible Dementia; Dementias, Transmissible; Spongiform Encephalopathies, Transmissible
Networked: 5345 relevant articles (109 outcomes, 360 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Neurodegenerative Diseases (Neurodegenerative Disease)
2. Alzheimer Disease (Alzheimer's Disease)
3. Infections
4. Parkinson Disease (Parkinson's Disease)
5. Huntington Disease (Huntington's Disease)

Experts

1. Collinge, John: 121 articles (10/2022 - 01/2002)
2. Aguzzi, Adriano: 101 articles (01/2022 - 10/2000)
3. Legname, Giuseppe: 68 articles (06/2022 - 05/2003)
4. Dong, Xiao-Ping: 68 articles (01/2022 - 08/2005)
5. Shi, Qi: 63 articles (01/2022 - 09/2006)
6. Caughey, Byron: 62 articles (01/2022 - 03/2002)
7. Gambetti, Pierluigi: 59 articles (10/2022 - 09/2002)
8. Mead, Simon: 59 articles (10/2022 - 04/2003)
9. Zerr, Inga: 58 articles (01/2022 - 11/2004)
10. Soto, Claudio: 54 articles (01/2021 - 08/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Prion Diseases:
1. PrionsIBA
2. Prion ProteinsIBA
3. Proteins (Proteins, Gene)FDA Link
4. Protein Isoforms (Isoforms)IBA
5. AntibodiesIBA
6. Biomarkers (Surrogate Marker)IBA
7. Quinacrine (Mepacrine)IBA
8. Amyloid (Amyloid Fibrils)IBA
9. CopperIBA
10. VaccinesIBA

Therapies and Procedures

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