TSES
MCID: PRN023
MIFTS: 71

Prion Disease (TSES) malady

Neuronal diseases, Cardiovascular diseases categories

Summaries for Prion Disease

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21Genetics Home Reference, 33MedlinePlus, 43NINDS, 63Wikipedia, 32MalaCards
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MedlinePlus:33 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 genetic prion diseases and brain disease. 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. Affiliated tissues include brain, cortex and spleen, and related mouse phenotypes are other and endocrine/exocrine gland.

Genetics Home Reference:21 Prion disease represents a group of conditions that affect the nervous system in humans and animals. In people, these conditions impair brain function, causing changes in memory, personality, and behavior; a decline in intellectual function (dementia); and abnormal movements, particularly difficulty with coordinating movements (ataxia). The signs and symptoms of prion disease typically begin in adulthood and worsen with time, leading to death within a few months to several years.

NINDS:43 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:63 Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of... more...

Aliases & Classifications for Prion Disease

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Sources:
8Disease Ontology, 9diseasecard, 63Wikipedia, 21Genetics Home Reference, 43NINDS, 10DISEASES, 44Novoseek, 48Orphanet, 33MedlinePlus, 60UMLS, 39NCIt, 56SNOMED-CT, 34MeSH, 26ICD10 via Orphanet, 25ICD10
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Classifications:

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


Characteristics (Orphanet epidemiological data):

48
prion disease:
Inheritance: Autosomal dominant,Sporadic; Prevalence: 1-9/1000000; Age of onset: Adulthood


Aliases & Descriptions:

prion disease 8 63 21 10 48
brain diseases 63 44 33 60
transmissible spongiform encephalopathies 63 21 43
prion diseases 43 44 60
inherited human transmissible spongiform encephalopathies 63 21
creutzfeldt-jakob disease 63 60
transmissible dementias 63 21
tses 63 21
human transmissible spongiform encephalopathies, inherited 60
transmissible spongiform encephalopathy 48
cerebral dysfunction syndrome 60
prion-associated disorders 21
spongiform encephalopathy 8
prion-induced disorders 21
prion protein diseases 21
prion induced disorder 8
prion disease pathway 8
prion protein disease 8
brain dysfunction 44
brain disease 10
prion protein 9


External Ids:

Disease Ontology8 DOID:649
MeSH34 D017096
ICD10 via Orphanet26 A81.0, A81.1, A81.8 A81.9, more

Related Diseases for Prion Disease

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Sources:
17GeneCards, 18GeneDecks
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Diseases related to Prion Disease via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 283)
idRelated DiseaseScoreTop Affiliating Genes
1genetic prion diseases31.1PRNP
2brain disease31.0PRNP, PSEN1, APP, MAPT, ITIH4
3scrapie30.8MSMB, CLU, MAPT, PRNP, PRND, RPSA
4familial creutzfeldt-jakob disease30.7MSMB, PRNP, APOE, PRND
5alzheimer's disease30.7ITIH4, PSEN2, APP, RECK, CLU, FPR2
6bovine spongiform encephalopathy30.6PRND, PRNP
7fatal familial insomnia30.6PRNP
8chronic wasting disease30.6PRNP
9creutzfeldt-jakob syndrome30.6PRNP, MAPT, MSMB, CST3, APP, APOE
10myositis30.6PRNP, PSEN1, APP, MAPT
11cerebral amyloid angiopathy30.5PRNP, MAPT, APP, CST3, PSEN1, APOE
12inclusion body myositis30.5MAPT, APP
13neuroblastoma30.4PRNP
14gerstmann-straussler-scheinker disease30.4PRNP, APP
15dementia30.4APOE, APP, CST3, PSEN2, PRNP, MAPT
16colorectal cancer30.3CST3, RECK, CLU
17cataract30.3GSS
18arthritis30.3CLU
19tauopathy30.3MAPT, APP, PSEN1
20rheumatoid arthritis30.3HLA-DQB1, CLU, FPR2, CST3, RECK
21schizophrenia30.2PSEN1, HLA-DQB1, SNAP25
22ischemia30.2CST3, PSEN1, MAPT, CLU
23amyloidosis30.2APOE, APP, CST3, PSEN1, PSEN2, CLU
24breast cancer30.2CST3, RECK, CLU, RPSA, MAPT, PSEN2
25hypertension30.1APOE, CST3
26myopathy30.1PRNP, APP
27encephalitis30.1ITIH4, APP
28parkinson's disease30.1MAPT, APOE
29amyotrophic lateral sclerosis30.1MAPT, CST3, APP, PSEN1, PRNP
30obesity30.1APOE
31multiple sclerosis30.1HLA-DQB1, APOE, CLU, CST3
32cerebrovascular disease30.1CST3, APP, RECK, APOE
33vascular dementia30.0APOE
34aphasia30.0MAPT
35herpes simplex30.0RECK
36leukemia30.0FPR1, ITIH4, RECK, F8
37down syndrome30.0APP, APOE, PSEN1, PSEN2, SNAP25, MAPT
38adenocarcinoma30.0MSMB, CLU, RECK, MAPT, PSEN2, RPSA
39wilson disease30.0PRNP
40atherosclerosis30.0RECK, CST3, CLU, PRNP, APOE
41huntington disease-like 129.9PRNP
42frontotemporal dementia29.9MAPT, PSEN1
43pick's disease29.9PSEN1, MAPT
44vascular disease29.9F8
45malignant glioma29.9ITIH4, APP, PSEN1, HLA-DQB1, PRNP, MAPT
46hydrocephalus29.8MAPT
47hepatitis29.6F8
48lung cancer29.6APP
49progressive supranuclear palsy29.6APP, MAPT, APOE, PRNP
50neuronitis10.5

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



Diseases related to prion disease

Clinical Features for Prion Disease

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Drugs & Therapeutics for Prion Disease

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Prion Disease

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Anatomical Context for Prion Disease

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32MalaCards
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MalaCards organs/tissues related to Prion Disease:

32
Brain, Cortex, Spleen, Skeletal muscle, Appendix, T cells, Cerebellum, Testes, Tonsil, Bone, Adrenal gland, Small intestine, Thalamus, Retina, Pituitary, B cells, Testis, Heart, Eye, Kidney, Spinal cord, Skin, Occipital lobe

Animal Models for Prion Disease or affiliated genes

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36MGI
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Publications for Prion Disease

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50PubMed
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Articles related to Prion Disease:

(show top 50)    (show all 803)
idTitleAuthorsYear
1
Chronic wasting disease in bank voles: characterisation of the shortest incubation time model for prion diseases. (23505374)
2013
2
Dysfunction of the PI3K-Akt-GSK-3 pathway is a common feature in cell culture and in vivo models of prion disease. (23741998)
2013
3
Immunohistochemical detection of disease-associated prion protein in the peripheral nervous system in experimental H-type bovine spongiform encephalopathy. (23269348)
2013
4
Relationships between clinicopathological features and cerebrospinal fluid biomarkers in Japanese patients with genetic prion diseases. (23555862)
2013
5
Human prion diseases: from kuru to variant creutzfeldt-jakob disease. (23225013)
2012
6
Genetic predictions of prion disease susceptibility in carnivore species based on variability of the prion gene coding region. (23236380)
2012
7
MicroRNA 146a (miR-146a) is over-expressed during prion disease and modulates the innate immune response and the microglial activation state. (22363497)
2012
8
Rescuing neurons in prion disease. (21158739)
2011
9
Detection of chronic wasting disease prions in salivary, urinary, and intestinal tissues of deer: potential mechanisms of prion shedding and transmission. (21525361)
2011
10
Correlation of polydispersed prion protein and characteristic pathology in the thalamus in variant Creutzfeldt-Jakob disease: implication of small oligomeric species. (21029243)
2011
11
Prion protein self-interaction in prion disease therapy approaches. (22029882)
2011
12
PET of brain prion protein amyloid in Gerstmann-StrAoussler-Scheinker disease. (19725833)
2010
13
Fatal transmissible amyloid encephalopathy: a new type of prion disease associated with lack of prion protein membrane anchoring. (20221436)
2010
14
Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein. (20610667)
2010
15
Genomic and post-genomic analyses of human prion diseases. (19566915)
2009
16
Infectious prions in pre-clinical deer and transmission of chronic wasting disease solely by environmental exposure. (19529769)
2009
17
Clinical and therapeutic aspects of prion disease. (18631792)
2008
18
Prion: disease or relief? (18758490)
2008
19
Disease-associated prion protein is not detectable in human systemic amyloid deposits. (17955450)
2007
20
Morphological changes and prion accumulation in the cerebellar cortex in Creutzfeldt-Jacob disease]. (18290372)
2007
21
Mechanistic insights into the cure of prion disease by novel antiprion compounds. (17652397)
2007
22
Comparison of CR36, a new heparan mimetic, and pentosan polysulfate in the treatment of prion diseases. (17325382)
2007
23
Modelling neurodegeneration in prion disease - applications for drug development. (23488996)
2007
24
Resistance to chronic wasting disease in transgenic mice expressing a naturally occurring allelic variant of deer prion protein. (17314157)
2007
25
Prions in skeletal muscles of deer with chronic wasting disease. (16439622)
2006
26
Human prion disease and relative risk associated with chronic wasting disease. (17176567)
2006
27
Prion protein polymorphisms in white-tailed deer influence susceptibility to chronic wasting disease. (16760415)
2006
28
The human prion gene M129V polymorphism is not associated with idiopathic Parkinson's disease in three distinct populations. (16298483)
2006
29
Neurodegeneration and oxidative stress: prion disease results from loss of antioxidant defence. (16416388)
2005
30
Anchors away--of plaques and pathology in prion disease. (16162891)
2005
31
Inhibition of P53-related apoptosis had no effect on PrP(Sc) accumulation and prion disease incubation time. (15686956)
2005
32
Prion disease and Alzheimer's disease: pathogenic overlap. (15190676)
2004
33
Uptake and efflux of quinacrine, a candidate for the treatment of prion diseases, at the blood-brain barrier. (15176436)
2004
34
Complete Freund's adjuvant immunization prolongs survival in experimental prion disease in mice. (12503092)
2003
35
Disease-associated F198S mutation increases the propensity of the recombinant prion protein for conformational conversion to scrapie-like form. (12372829)
2002
36
Plasminogen binds to disease-associated prion protein of multiple species. (11438139)
2001
37
Familial Creutzfeldt-Jakob disease associated with a point mutation at codon 210 of the prion protein gene. (11733840)
2001
38
A transgenic model of a familial prion disease. (11214912)
2000
39
Familial Creutzfeldt-Jakob disease in a patient carrying both a presenilin 1 missense substitution and a prion protein gene insertion. (10896268)
2000
40
Familial Creutzfeldt-Jakob disease with a novel 120-bp insertion in the prion protein gene (10553985)
1999
41
The use of monoclonal antibodies in human prion disease. (10360268)
1999
42
Doxycycline control of prion protein transgene expression modulates prion disease in mice. (9770528)
1998
43
Role of microglia in neuronal cell death in prion disease. (9669696)
1998
44
Proteinase-K-resistant prion protein isoforms in Gerstmann-StrAoussler-Scheinker disease (Indiana kindred). (8939199)
1996
45
The genetics and transgenetics of human prion disease. (8030945)
1994
46
Familial Pick's disease and dementia in frontal lobe degeneration of non-Alzheimer type are not variants of prion disease. (8006666)
1994
47
Prion disease scare. (8371765)
1993
48
Inherited prion disease. (8490359)
1993
49
Presence of prion protein in peripheral tissues of Libyan Jews with Creutzfeldt-Jakob disease. (1352391)
1992
50
Diffuse deposition of immunohistochemically labeled prion protein in the granular layer of the cerebellum in a patient with Creutzfeldt-Jakob disease. (1686132)
1991

Genetic Variations for Prion Disease

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Expression for genes affiliated with Prion Disease

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1BioGPS, 15Gene Expression Omnibus DataSets
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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

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59Tocris Bioscience, 52R&D Systems, 29KEGG, 37NCBI BioSystems Database, 53Reactome, 51QIAGEN, 4Cell Signaling Technology
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Compounds for genes affiliated with Prion Disease

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59Tocris Bioscience, 28IUPHAR, 44Novoseek, 11DrugBank, 24HMDB, 49PharmGKB, 2BitterDB
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Compounds related to Prion Disease according to GeneCards/GeneDecks:

(show top 50)    (show all 111)
idCompoundScoreTop Affiliating Genes
1wrw45910.8FPR3, FPR2, FPR1
2wkymvm59 2811.8FPR2, FPR3, FPR1
3boc-mlf5910.8FPR2, FPR3, FPR1
4annexin i-(2-26)2810.8FPR1, FPR3, FPR2
5fmet-met-tyr-ala-leu-phe2810.8FPR1, FPR3, FPR2
6mmk 15910.8FPR1, FPR2, FPR3
7biotin44 11 2412.8PSEN1, GSS, PSEN2, ITIH4, FPR1, APOE
8vitamin b124410.8APOE, PSEN1, MAPT, CST3, FPR1
9h2o24410.8FPR1, CLU, MAPT, PRNP, ITIH4, PSEN2
10thioflavine s4410.8APP, MAPT, APOE
11valine4410.8PSEN1, APOE, PRND, MAPT, APP, PRNP
12methionine4410.7PSEN1, F8, PRNP, PRND, MAPT, RECK
13heparin44 28 11 2413.7F8, FPR1, ITIH4, CLU, RECK, MAPT
14folate4410.7APOE, CST3, APP, PSEN1, MAPT, RECK
15serine4410.7F8, PSEN2, GSS, FPR1, CLU, MAPT
16cyclosporin a44 28 5912.7APOE, F8, RECK, APP, ITIH4, CST3
17superoxide44 2411.7CLU, FPR2, PRNP, PSEN1, GSS, FPR1
18lactacystin4410.7APP, MAPT, PSEN2, PSEN1, APOE
19glutamine4410.7HLA-DQB1, CST3, APP, APOE, PSEN1, MAPT
20sodium dodecylsulfate4410.7MAPT, APP, APOE, ITIH4, PRNP
21formate4410.7APOE, APP, PRNP, MAPT
22homocysteine44 2411.7CLU, PSEN1, APOE, F8, CST3
23lactate4410.7CLU, RECK, ITIH4, F8, PSEN2, RPSA
24tacrine44 1111.7APP, MAPT, APOE
25alanine4410.7FPR1, APOE, PSEN1, F8, ITIH4, PRNP
26cholesterol44 28 11 2413.7CST3, APP, PSEN1, PSEN2, F8, SNAP25
27aspartate4410.7PSEN1, CST3, APP, MAPT, F8, HLA-DQB1
28glutamate4410.7APP, APOE, PSEN2, HLA-DQB1, SNAP25, CLU
29arginine4410.7APOE, PSEN1, APP, RECK, PRNP, ITIH4
30polysaccharide4410.7PRNP, F8, ITIH4, CLU, RECK
31n acetylcysteine4410.7CLU, FPR1, MAPT, GSS, APOE, CST3
32tyrosine4410.7FPR2, CLU, HLA-DQB1, F8, PSEN2, FPR1
33donepezil44 11 2412.7MAPT, APP, APOE
34lipid4410.6RECK, SNAP25, PSEN1, APOE, APP, CST3
35vegf4410.6CST3, APOE, APP, PSEN2, F8, RECK
36memantine44 28 1112.6APP, ITIH4, MAPT
37paraffin4410.6CLU, MAPT, PRNP, ITIH4, APOE, PSEN2
38actinomycin d4410.6CLU, MAPT, ITIH4, PSEN2, APOE, APP
39cysteine4410.6CLU, MAPT, ITIH4, F8, GSS, MSMB
40calcium44 49 11 2413.6MSMB, FPR3, FPR1, CLU, RECK, PRNP
41oligonucleotide4410.6FPR2, F8, HLA-DQB1, CLU, PSEN1, FPR1
42sucrose44 11 2412.6SNAP25, F8, PRNP, RECK, FPR1
43tunicamycin44 5911.6APP, ITIH4, RECK, CLU
44iron44 2411.5APP, CST3, RECK, PRNP, SNAP25, F8
454-hydroxynonenal44 2411.4PSEN1, APOE, APP, MAPT
46dopamine44 28 11 2413.4CLU, MAPT, ITIH4, SNAP25, APOE, APP
47galantamine44 49 1112.4APOE, MAPT, APP
48silver4410.4CST3, F8, RECK, MAPT
49amino acid4410.3PRNP, F8, ITIH4
50chloroquine44 2 49 28 1114.1ITIH4, MAPT, APP, APOE

GO Terms for genes affiliated with Prion Disease

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16Gene Ontology
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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

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  • Antibodies
  • Proteins
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Sources for Prion Disease

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet