TSES
MCID: PRN023
MIFTS: 60

Prion Disease (TSES) malady

Neuronal, Cardiovascular categories

Summaries for Prion Disease

Sources:
21Genetics Home Reference, 34MedlinePlus, 44NINDS, 64Wikipedia, 33MalaCards
See all sources

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MedlinePlus:34 The brain is the control center of the body. it controls thoughts, memory, speech, and movement. it regulates the function of many organs. when the brain is healthy, it works quickly and automatically. however, when problems occur, the results can be devastating. inflammation in the brain can lead to problems such as vision loss, weakness and paralysis. loss of brain cells, which happens if you suffer a stroke, can affect your ability to think clearly. brain tumors can also press on nerves and affect brain function. some brain diseases are genetic. and we do not know what causes some brain diseases, such as alzheimer's disease. the symptoms of brain diseases vary widely depending on the specific problem. in some cases, damage is permanent. in other cases, treatments such as surgery, medicines, or physical therapy can correct the source of the problem or improve symptoms.

MalaCards: Prion Disease, also known as brain diseases, is related to brain disease and genetic prion diseases. An important gene associated with Prion Disease is FPR2 (formyl peptide receptor 2), and among its related pathways are Notch Pathway and A-beta Uptake & Degradation. The compounds wrw4 and wkymvm have been mentioned in the context of this disorder. Related mouse phenotypes are other and endocrine/exocrine gland.

Genetics Home Reference:21 Prion diseases belong to group of progressive conditions that affect the nervous system in humans and animals. In people, prion diseases impair brain function, causing memory changes, personality changes, a decline in intellectual function (dementia), and problems with movement that worsen over time. The signs and symptoms of these conditions typically begin in adulthood, and these disorders lead to death within a few months to several years.

NINDS:44 Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of rare degenerative brain disorders characterized by tiny holes that give the brain a "spongy" appearance. These holes can be seen when brain tissue is viewed under a microscope.

Wikipedia:64 Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of... more...

Aliases & Classifications for Prion Disease

Sources:
8Disease Ontology, 9diseasecard, 64Wikipedia, 21Genetics Home Reference, 44NINDS, 10DISEASES, 45Novoseek, 49Orphanet, 34MedlinePlus, 61UMLS, 40NCIt, 57SNOMED-CT, 35MeSH, 26ICD10 via Orphanet, 25ICD10
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Neuronal, Cardiovascular


Characteristics (Orphanet epidemiological data):

49
prion disease:
Inheritance: Autosomal dominant,Sporadic; Prevalence: 1-9/1000000; Age of onset: Adulthood


Aliases & Descriptions:

prion disease 8 64 21 10 49
brain diseases 64 45 34 61
transmissible spongiform encephalopathies 64 21 44
prion diseases 44 45 61
inherited human transmissible spongiform encephalopathies 64 21
creutzfeldt-jakob disease 64 61
transmissible dementias 64 21
tses 64 21
human transmissible spongiform encephalopathies, inherited 61
transmissible spongiform encephalopathy 49
cerebral dysfunction syndrome 61
prion-associated disorders 21
spongiform encephalopathy 8
prion-induced disorders 21
prion induced disorder 8
prion protein diseases 21
prion disease pathway 8
prion protein disease 8
brain dysfunction 45
brain disease 10
prion protein 9


External Ids:

Disease Ontology8 DOID:649
MeSH35 D017096
ICD10 via Orphanet26 A81

Related Diseases for Prion Disease

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the Prion Disease family:

prion disease with protracted course

Diseases related to Prion Disease via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 274)
idRelated DiseaseScoreTop Affiliating Genes
1brain disease31.1MAPT, PRNP, ITIH4, PSEN1, APP
2genetic prion diseases30.9PRNP
3scrapie30.9RPSA, CST3, PRND, PRNP, MAPT, CLU
4bovine spongiform encephalopathy30.7PRND, PRNP
5fatal familial insomnia30.7PRNP
6chronic wasting disease30.7PRNP
7creutzfeldt-jakob syndrome30.6CST3, APP, APOE, SNAP25, PRND, PRNP
8cerebral amyloid angiopathy30.6CST3, APP, APOE, PSEN1, PRNP, MAPT
9inclusion body myositis30.5MAPT, APP
10gerstmann-straussler-scheinker disease30.4APP, PRNP
11colorectal cancer30.4CLU, RECK, CST3
12pick's disease30.3PSEN1, MAPT
13arthritis30.3CLU
14tauopathy30.3MAPT, PSEN1, APP
15amyloidosis30.2CST3, APP, APOE, PSEN1, PSEN2, PRNP
16parkinson's disease30.2MAPT, APOE
17protein s deficiency30.2F8
18amyotrophic lateral sclerosis30.2CST3, APP, PSEN1, PRNP, MAPT
19atherosclerosis30.0CST3, APOE, PRNP, RECK, CLU
20vascular dementia30.0APOE
21aphasia30.0MAPT
22memory impairment30.0APP, APOE, PSEN1, ITIH4, PRNP, MAPT
23adenocarcinoma30.0MSMB, CLU, RECK, MAPT, PSEN2, RPSA
24wilson disease30.0PRNP
25familial creutzfeldt-jakob disease30.0APOE, PRND, PRNP, MSMB
26huntington disease-like 130.0PRNP
27frontotemporal dementia30.0PSEN1, MAPT
28vascular disease30.0F8
29alzheimer's disease30.0PSEN2, PSEN1, APOE, APP, CST3, SNAP25
30blindness29.7CLU
31progressive supranuclear palsy29.7APP, APOE, PRNP, MAPT
32variant creutzfeldt-jakob disease10.5
33insomnia10.4
34tropical spastic paraparesis10.4
35status epilepticus10.4
36akinetic mutism10.4
37mutism10.4
38myoclonus10.4
39blood protein disease10.4
40gerstmann syndrome10.4
41muscle eye brain disease10.3
42lip disease10.3
43hypoxia10.3
44hemoglobin d disease10.2
45wernicke-korsakoff syndrome10.2
46hemoglobin c disease10.2
47alpers syndrome10.2
48familial mediterranean fever10.2
49alexia10.2
50meningoencephalitis10.2

Graphical network of the top 20 diseases related to Prion Disease:



Diseases related to prion disease

Clinical Features for Prion Disease

Drugs & Therapeutics for Prion Disease

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Prion Disease

Drug clinical trials:

Search ClinicalTrials for Prion Disease

Search NIH Clinical Center for Prion Disease

Search CenterWatch for Prion Disease

Genetic Tests for Prion Disease

Anatomical Context for Prion Disease

Animal Models for Prion Disease or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

Publications for Prion Disease

Sources:
51PubMed
See all sources

Articles related to Prion Disease:

(show top 50)    (show all 805)
idTitleAuthorsYear
1
Ethics in prion disease. (23906487)
2013
2
Prion-seeding activity in cerebrospinal fluid of deer with chronic wasting disease. (24282599)
2013
3
Immunotherapeutic approaches in prion disease: progress, challenges and potential directions. (23647278)
2013
4
How structure shapes (dys)function: a perspective to understanding brain region-specific degeneration in prion disease. (23924581)
2013
5
Amyloid-I^ induced signaling by cellular prion protein and Fyn kinase in Alzheimer disease. (22987042)
2013
6
Hereditary form of prion disease in Poland. (23319218)
2012
7
Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA. (22744790)
2012
8
Distinct neuropsychological profiles correspond to distribution of cortical thinning in inherited prion disease caused by insertional mutation. (21849340)
2012
9
From high-throughput cell culture screening to mouse model: identification of new inhibitor classes against prion disease. (21755599)
2011
10
Can prion disease suspicion be supported earlier? Clinical, radiological and laboratory findings in a series of cases. (21869605)
2011
11
Early behavioral changes and quantitative analysis of neuropathological features in murine prion disease: stereological analysis in the albino Swiss mice model. (21862877)
2011
12
Unaltered prion protein expression in Alzheimer disease patients. (21654203)
2011
13
Using qualitative methods to investigate risk perception of Canadian medical laboratory workers in relation to current prion disease infection control policies. (21218349)
2011
14
Chronic wasting disease prion trafficking via the autonomic nervous system. (21777560)
2011
15
Codon 129 polymorphism and the E200K mutation do not affect the cellular prion protein isoform composition in the cerebrospinal fluid from patients with Creutzfeldt-Jakob disease. (20529115)
2010
16
Prions: protein aggregation and infectious diseases. (19789378)
2009
17
Prion disease causes less severe lesions in human hippocampus than other parts of brain. (18588585)
2008
18
Association between deposition of beta-amyloid and pathological prion protein in sporadic Creutzfeldt-Jakob disease. (18349519)
2008
19
Induced neuroprotection independently from PrPSc accumulation in a mouse model for prion disease treated with simvastatin. (18541796)
2008
20
Neuroimaging findings in human prion disease. (17135459)
2007
21
Chronic wasting disease of elk and deer and Creutzfeldt-Jakob disease: comparative analysis of the scrapie prion protein. (16338930)
2006
22
Myiasis as a risk factor for prion diseases in humans. (16987255)
2006
23
Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. (16606639)
2006
24
Analysis of the cerebellar proteome in a transgenic mouse model of inherited prion disease reveals preclinical alteration of calcineurin activity. (16572473)
2006
25
Biochemical fingerprints of prion diseases: scrapie prion protein in human prion diseases that share prion genotype and type. (15606903)
2005
26
Amyloid associated proteins in Alzheimer's and prion disease. (15975027)
2005
27
Prion disease incubation time is not affected in mice heterozygous for a dynein mutation. (15567146)
2005
28
Specificity of lymphoreticular accumulation of prion protein for variant Creutzfeldt-Jakob disease. (14990604)
2004
29
Prion protein heterogeneity in sporadic but not variant Creutzfeldt-Jakob disease: UK cases 1991-2002. (15174020)
2004
30
Role of interleukin-1 in prion disease-associated astrocyte activation. (15277240)
2004
31
The residue 129 polymorphism in human prion protein does not confer susceptibility to Creutzfeldt-Jakob disease by altering the structure or global stability of PrPC. (15123682)
2004
32
Pentosan polysulfate as a prophylactic and therapeutic agent against prion disease. (12789602)
2003
33
Sporadic Creutzfeldt-Jakob disease with MM1-type prion protein and plaques. (12578942)
2003
34
Is there a risk of prion disease after the administration of urinary-derived gonadotrophins? (12093821)
2002
35
Prion disease: close encounters of the cellular kind. (11937041)
2002
36
Cellular phenotyping of secretory and nuclear prion proteins associated with inherited prion diseases. (11756421)
2002
37
Mad deer. The North American version of prion disease. (12475922)
2002
38
Central nervous system inflammation and prion disease pathogenesis. (21374504)
2001
39
Influence of the prion protein and the apolipoprotein E genotype on the Creutzfeldt-Jakob Disease phenotype. (11684342)
2001
40
Absence of protease-resistant prion protein in the cerebrospinal fluid of Creutzfeldt-Jakob disease. (11329135)
2001
41
Prion protein deposition and abnormal synaptic protein expression in the cerebellum in Creutzfeldt-Jakob disease. (10842016)
2000
42
Evidence for oxidative stress in experimental prion disease. (10964599)
2000
43
New haplotype of familial Creutzfeldt-Jakob disease with a codon 200 mutation and a codon 219 polymorphism of the prion protein gene in a Japanese family. (10672318)
2000
44
Effectiveness of anthracycline against experimental prion disease in Syrian hamsters. (9148807)
1997
45
Identification of the prion protein allotypes which accumulate in the brain of sporadic and familial Creutzfeldt-Jakob disease patients. (9142120)
1997
46
Synthetic growth hormone has no inducting effect in the development of a prion disease: an experimental study on the scrapie model in hamsters. (9415545)
1997
47
Fatal familial insomnia and familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphism. (7908444)
1994
48
Molecular genetics of prion diseases in France. French Research Group on Epidemiology of Human Spongiform Encephalopathies. (7991124)
1994
49
Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Straussler syndrome. (8461023)
1993
50
Prion diseases in humans and their relevance to other neurodegenerative diseases. (8401789)
1993

Genetic Variations for Prion Disease

Expression for genes affiliated with Prion Disease

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Prion Disease

Search GEO for disease gene expression data for Prion Disease.

Pathways for genes affiliated with Prion Disease

Sources:
60Tocris Bioscience, 53R&D Systems, 30KEGG, 38NCBI BioSystems Database, 54Reactome, 52QIAGEN, 4Cell Signaling Technology
See all sources

Compounds for genes affiliated with Prion Disease

Sources:
60Tocris Bioscience, 29IUPHAR, 45Novoseek, 11DrugBank, 24HMDB, 50PharmGKB, 2BitterDB
See all sources

Compounds related to Prion Disease according to GeneCards/GeneDecks:

(show top 50)    (show all 111)
idCompoundScoreTop Affiliating Genes
1wrw46010.8FPR3, FPR2, FPR1
2wkymvm60 2911.8FPR2, FPR3, FPR1
3boc-mlf6010.8FPR2, FPR3, FPR1
4annexin i-(2-26)2910.8FPR1, FPR3, FPR2
5fmet-met-tyr-ala-leu-phe2910.8FPR1, FPR3, FPR2
6mmk 16010.8FPR1, FPR2, FPR3
7biotin45 11 2412.8PSEN1, GSS, PSEN2, ITIH4, FPR1, APOE
8vitamin b124510.8APOE, PSEN1, MAPT, CST3, FPR1
9h2o24510.8FPR1, CLU, MAPT, PRNP, ITIH4, PSEN2
10thioflavine s4510.8APP, MAPT, APOE
11valine4510.8PSEN1, APOE, PRND, MAPT, APP, PRNP
12methionine4510.7PSEN1, F8, PRNP, PRND, MAPT, RECK
13heparin45 29 11 2413.7F8, FPR1, ITIH4, CLU, RECK, MAPT
14folate4510.7APOE, CST3, APP, PSEN1, MAPT, RECK
15serine4510.7F8, PSEN2, GSS, FPR1, CLU, MAPT
16cyclosporin a45 29 6012.7APOE, F8, RECK, APP, ITIH4, CST3
17superoxide45 2411.7CLU, FPR2, PRNP, PSEN1, GSS, FPR1
18lactacystin4510.7APP, MAPT, PSEN2, PSEN1, APOE
19glutamine4510.7HLA-DQB1, CST3, APP, APOE, PSEN1, MAPT
20sodium dodecylsulfate4510.7MAPT, APP, APOE, ITIH4, PRNP
21formate4510.7APOE, APP, PRNP, MAPT
22homocysteine45 2411.7CLU, PSEN1, APOE, F8, CST3
23lactate4510.7CLU, RECK, ITIH4, F8, PSEN2, RPSA
24tacrine45 1111.7APP, MAPT, APOE
25alanine4510.7FPR1, APOE, PSEN1, F8, ITIH4, PRNP
26cholesterol45 29 11 2413.7CST3, APP, PSEN1, PSEN2, F8, SNAP25
27aspartate4510.7PSEN1, CST3, APP, MAPT, F8, HLA-DQB1
28glutamate4510.7APP, APOE, PSEN2, HLA-DQB1, SNAP25, CLU
29arginine4510.7APOE, PSEN1, APP, RECK, PRNP, ITIH4
30polysaccharide4510.7PRNP, F8, ITIH4, CLU, RECK
31n acetylcysteine4510.7CLU, FPR1, MAPT, GSS, APOE, CST3
32tyrosine4510.7FPR2, CLU, HLA-DQB1, F8, PSEN2, FPR1
33donepezil45 11 2412.7MAPT, APP, APOE
34lipid4510.6RECK, SNAP25, PSEN1, APOE, APP, CST3
35vegf4510.6CST3, APOE, APP, PSEN2, F8, RECK
36memantine45 29 1112.6APP, ITIH4, MAPT
37paraffin4510.6CLU, MAPT, PRNP, ITIH4, APOE, PSEN2
38actinomycin d4510.6CLU, MAPT, ITIH4, PSEN2, APOE, APP
39cysteine4510.6CLU, MAPT, ITIH4, F8, GSS, MSMB
40calcium45 50 11 2413.6MSMB, FPR3, FPR1, CLU, RECK, PRNP
41oligonucleotide4510.6FPR2, F8, HLA-DQB1, CLU, PSEN1, FPR1
42sucrose45 11 2412.6SNAP25, F8, PRNP, RECK, FPR1
43tunicamycin45 6011.6APP, ITIH4, RECK, CLU
44iron45 2411.5APP, CST3, RECK, PRNP, SNAP25, F8
454-hydroxynonenal45 2411.4PSEN1, APOE, APP, MAPT
46dopamine45 29 11 2413.4CLU, MAPT, ITIH4, SNAP25, APOE, APP
47galantamine45 50 1112.4APOE, MAPT, APP
48silver4510.4CST3, F8, RECK, MAPT
49amino acid4510.3PRNP, F8, ITIH4
50chloroquine45 2 50 29 1114.1ITIH4, MAPT, APP, APOE

GO Terms for genes affiliated with Prion Disease

Sources:
16Gene Ontology
See all sources

Cellular components related to Prion Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1ciliary rootletGO:03525310.6PSEN1, APP, PSEN2
2growth coneGO:03042610.5MAPT, SNAP25, PSEN1, PSEN2
3dendritic shaftGO:04319810.5PSEN2, PSEN1, APP
4neuromuscular junctionGO:03159410.5PSEN2, PSEN1, APP
5anchored to membraneGO:03122510.4RECK, PRNP, PRND
6axonGO:03042410.4PSEN2, MAPT, PSEN1, APP
7plasma membraneGO:00588610.3FPR2, RPSA, APP, APOE, PSEN2, F8
8platelet alpha granule lumenGO:03109310.3F8, CLU, APP
9perinuclear region of cytoplasmGO:04847110.1PSEN1, PSEN2, SNAP25, CLU, APP

Biological processes related to Prion Disease according to GeneCards/GeneDecks:

(show all 21)
idNameGO IDScoreTop Affiliating Genes
1cellular copper ion homeostasisGO:00687810.8PRNP, PRND, APP
2positive regulation of coagulationGO:05082010.7PSEN1, PSEN2
3smooth endoplasmic reticulum calcium ion homeostasisGO:05156310.7APP, PSEN1
4regulation of protein bindingGO:04339310.7PSEN2, PSEN1, APP
5myeloid leukocyte differentiationGO:00257310.7PSEN2, PSEN1
6response to oxidative stressGO:00697910.7APP, PSEN1, PRNP, GSS
7regulation of epidermal growth factor-activated receptor activityGO:00717610.7APP, PSEN2
8T cell activation involved in immune responseGO:00228610.7PSEN1, PSEN2
9endoplasmic reticulum calcium ion homeostasisGO:03246910.7PSEN2, PSEN1
10amyloid precursor protein catabolic processGO:04298710.7PSEN2, PSEN1
11beta-amyloid metabolic processGO:05043510.7PSEN1, PSEN2
12axon cargo transportGO:00808810.6MAPT, APP
13anagenGO:04264010.6PSEN1, PSEN2
14platelet degranulationGO:00257610.6CLU, F8, APP
15T cell receptor signaling pathwayGO:05085210.6HLA-DQB1, PSEN2, PSEN1
16cellular component movementGO:00692810.5FPR1, FPR3, FPR2
17brain morphogenesisGO:04885410.5PSEN2, PSEN1
18dorsal/ventral neural tube patterningGO:02190410.4PSEN2, PSEN1
19cell fate specificationGO:00170810.4PSEN1, PSEN2
20nitric oxide mediated signal transductionGO:00726310.3FPR1, APOE
21hematopoietic progenitor cell differentiationGO:00224410.1PSEN1, PSEN2

Molecular functions related to Prion Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1N-formyl peptide receptor activityGO:00498210.5FPR1, FPR2, FPR3
2endopeptidase inhibitor activityGO:00486610.4RECK, ITIH4, CST3
3serine-type endopeptidase inhibitor activityGO:00486710.4APP, RECK, ITIH4
4lipoprotein particle bindingGO:07181310.3MAPT, APOE
5protein bindingGO:00551510.2MAPT, ITIH4, CLU, FPR1, SNAP25, F8
6copper ion bindingGO:00550710.1PRNP, PRND, F8

Products for genes affiliated with Prion Disease

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Prion Disease

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet