TSES
MCID: PRN023
MIFTS: 71

Prion Disease (TSES) malady

Neuronal diseases, Cardiovascular diseases categories

Summaries for Prion Disease

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Sources:
33MedlinePlus, 43NINDS, 21Genetics Home Reference, 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.

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.

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.

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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10DISEASES, 8Disease Ontology, 63Wikipedia, 21Genetics Home Reference, 48Orphanet, 60UMLS, 9diseasecard, 44Novoseek, 33MedlinePlus, 43NINDS, 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 induced disorder 8
prion protein diseases 21
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, Tonsil, Cerebellum, Bone, Testes, B cells, Occipital lobe, Thalamus, Eye, Testis, Skin, Adrenal gland, Kidney, Small intestine, Heart, Spinal cord, Retina, Pituitary

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
Multiparameter MR imaging in the 6-OPRI variant of inherited prion disease. (23538406)
2013
2
Amyloidosis, Synucleinopathy, and Prion Encephalopathy in a Neuropathic Lysosomal Storage Disease: The CNS-Biomarker Potential of Peripheral Blood. (24278249)
2013
3
Detection of disease-associated prion protein in the optic nerve and the adrenal gland of cattle with bovine spongiform encephalopathy by using highly sensitive immunolabeling procedures. (22260993)
2012
4
A Medicinal Herb Scutellaria lateriflora Inhibits PrP Replication in vitro and Delays the Onset of Prion Disease in Mice. (22363300)
2012
5
Altered Prion protein expression pattern in CSF as a biomarker for Creutzfeldt-Jakob disease. (22558368)
2012
6
Emerging prion disease drives host selection in a wildlife population. (22645831)
2012
7
The diverse roles of mononuclear phagocytes in prion disease pathogenesis. (22421209)
2012
8
Persistent retroviral infection with MoMuLV influences neuropathological signature and phenotype of prion disease. (22271154)
2012
9
Fatal prion disease in a mouse model of genetic E200K Creutzfeldt-Jakob disease. (22072968)
2011
10
Phenotypic variability of sporadic human prion disease and its molecular basis: past, present, and future. (21107851)
2011
11
Infectious prion protein alters manganese transport and neurotoxicity in a cell culture model of prion disease. (21871919)
2011
12
Pathologic evidence that the T188R mutation in PRNP is associated with prion disease. (21107135)
2010
13
Prion-like aggregates: infectious agents in human disease. (20870462)
2010
14
Sensitive discrimination and detection of prion disease-associated isoform with a dual-aptamer strategy by developing a sandwich structure of magnetic microparticles and quantum dots. (21038863)
2010
15
Rhythmic pupillary oscillation in Creutzfeldt-Jakob disease associated with the Glu/Lys mutation of prion protein codon 200. (20077484)
2010
16
Crystallographic studies of prion protein (PrP) segments suggest how structural changes encoded by polymorphism at residue 129 modulate susceptibility to human prion disease. (20685658)
2010
17
No evidence for prion protein gene locus multiplication in Creutzfeldt-Jakob disease. (20105449)
2010
18
Increased GH secretion in scrapie, a prion-associated neurodegenerative disease, is not due to suppressed IGF-1 negative feedback. (19179038)
2009
19
Shadoo (Sprn) and prion disease incubation time in mice. (19513788)
2009
20
A systems approach to prion disease. (19308092)
2009
21
Magnetic resonance diagnostic markers in clinically sporadic prion disease: a combined brain magnetic resonance imaging and spectroscopy study. (19755520)
2009
22
Neuroimaging in human prion disease: Searching in the mist. (21160720)
2009
23
A prion disease of cervids: chronic wasting disease. (18381058)
2008
24
Elevated manganese levels in blood and CNS in human prion disease. (18234506)
2008
25
Investigation of mcp1 as a quantitative trait gene for prion disease incubation time in mouse. (18716327)
2008
26
Phenotypic heterogeneity and genetic modification of P102L inherited prion disease in an international series. (18757886)
2008
27
Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients. (18751990)
2008
28
Heat shock factor 1 regulates lifespan as distinct from disease onset in prion disease. (18757733)
2008
29
Codon 129 polymorphism of prion protein gene in sporadic Alzheimer's disease. (18217885)
2008
30
Mutant prion protein D202N associated with familial prion disease is retained in the endoplasmic reticulum and forms 'curly' intracellular aggregates. (17873292)
2007
31
Immunisation strategies against prion diseases: prime-boost immunisation with a PrP DNA vaccine containing foreign helper T-cell epitopes does not prevent mouse scrapie. (17499458)
2007
32
Traffic of prion protein between different compartments on the neuronal surface, and the propagation of prion disease. (16884720)
2006
33
Phenotypic heterogeneity in inherited prion disease (P102L) is associated with differential propagation of protease-resistant wild-type and mutant prion protein. (16597650)
2006
34
"Strange things I have in head": evidence of prion disease in Shakespeare's Macbeth. (16355346)
2006
35
Prion disease: exponential growth requires membrane binding. (16581847)
2006
36
Creutzfeldt-Jakob disease (CJD) with a mutation at codon 148 of prion protein gene: relationship with sporadic CJD. (16314483)
2005
37
The effect of disease-associated mutations on the folding pathway of human prion protein. (14761942)
2004
38
Alterations of somatotropic function in prion disease in sheep. (15531730)
2004
39
Absence of association between codon 129/219 polymorphisms of the prion protein gene and Alzheimer's disease in Japan. (14520676)
2003
40
Laboratory and imaging studies for the diagnosis of prion disease]. (15152471)
2003
41
Immunization treatment approaches in Alzheimer's and prion diseases. (12169219)
2002
42
Transmission of prion disease. (12064258)
2002
43
Distribution of protease-resistant prion protein and spongiform encephalopathy in free-ranging mule deer (Odocoileus hemionus) with chronic wasting disease. (12243464)
2002
44
Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease. (11504948)
2001
45
Intracerebral distribution of the abnormal isoform of the prion protein in sporadic Creutzfeldt-Jakob disease and fatal insomnia. (10871544)
2000
46
Binding of disease-associated prion protein to plasminogen. (11100730)
2000
47
Possible retroviral origin of prion disease: could prion disease be reconsidered as a preleukemia syndrome? (10221168)
1999
48
Neuropathology of prion diseases. (10221164)
1999
49
Prion diseases and a new variant of Creutzfeldt-Jakob disease]. (9128415)
1996
50
Prion disease with 144 base pair insertion in a Japanese family line. (7572084)
1995

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

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:00498210.5FPR2, FPR3, FPR1
2endopeptidase inhibitor activityGO:00486610.4CST3, ITIH4, RECK
3serine-type endopeptidase inhibitor activityGO:00486710.4RECK, ITIH4, APP
4lipoprotein particle bindingGO:07181310.3MAPT, APOE
5protein bindingGO:00551510.2PSEN2, PSEN1, APOE, APP, CST3, RPSA
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