MCID: PRN023
MIFTS: 44

Prion Disease malady

Categories: Neuronal diseases, Mental diseases

Aliases & Classifications for Prion Disease

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Aliases & Descriptions for Prion Disease:

Name: Prion Disease 11 24 13
Prion Diseases 47 48 2 37 66
Prion Disease Pathway 11 66
Human Transmissible Spongiform Encephalopathies, Inherited 66
Inherited Human Transmissible Spongiform Encephalopathies 24
Transmissible Spongiform Encephalopathies 24
Transmissible Spongiform Encephalopathy 11
Prion-Associated Disorders 24
 
Spongiform Encephalopathy 11
Transmissible Dementias 24
Prion-Induced Disorders 24
Prion Induced Disorder 11
Prion Protein Diseases 24
Prion Protein Disease 11
Prion Protein 12
Tses 24

Classifications:



External Ids:

Disease Ontology11 DOID:649
ICD1028 A81.9
MeSH37 D017096
NCIt43 C27585

Summaries for Prion Disease

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Genetics Home Reference:24 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.

MalaCards based summary: Prion Disease, also known as prion diseases, is related to huntington disease-like 1 and prion disease with protracted course. An important gene associated with Prion Disease is PRNP (Prion Protein), and among its related pathways are SIDS Susceptibility Pathways and Oxidative Stress. Affiliated tissues include brain, cortex and endothelial, and related mouse phenotypes are immune system and hematopoietic system.

Disease Ontology:11 A brain disease that is characterized by brain damage resulting from the abnormal folding, clumping and accumulation of cellular proteins in the brain induced by prion proteins.

CDC:2 Classic CJD is a human prion disease. It is a neurodegenerative disorder with characteristic clinical and diagnostic features. 

NINDS:47 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:69 Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of... more...

Related Diseases for Prion Disease

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

(show top 50)    (show all 54)
idRelated DiseaseScoreTop Affiliating Genes
1huntington disease-like 131.9MSMB, PRNP
2prion disease with protracted course31.8ENO2, MSMB, PRND, PRNP, SPRN
3genetic prion diseases12.3
4familial alzheimer-like prion disease11.9
5creutzfeldt-jakob disease10.8
6kuru10.6
72q33.1 microdeletion syndrome10.6ENO2, MAP2
8ovarian mucinous cystadenocarcinoma10.4IL1B, MAP2, PRNP
9large intestine lipoma10.4ENO2, MAP2
10choroid plexus cancer10.4CR2, PRNP, SPRN
11periodic paralyses10.4ENO2, IL1B
12burns10.3IL1B, SOD2
13postinflammatory pulmonary fibrosis10.3CAT, TF
14scrapie10.3
15diabetes insipidus, neurohypophyseal10.3MSMB, PRNP
16encephalopathy10.3
17primary syphilis10.3ENO2, MAP2
18familial mediterranean fever type 210.3PRND, PRNP
19neuronitis10.3
20gerstmann-straussler disease10.2
21microsporidiosis10.2ENO2, PRNP
22gemistocytic astrocytoma10.2IL1B, PRNP, TF
23scn8a encephalopathy10.2IL1B, TF
24insomnia, fatal familial10.2
25chronic wasting disease10.2
26prediabetes syndrome10.2CAT, SOD2
27trochlear nerve neoplasm10.1ENO2, MAP2
28pseudoretinitis pigmentosa10.1CAT, IL1B, SOD2
29dementia10.0
30cerebritis10.0
31alzheimer disease9.9
32neuropathy9.9
33huriez syndrome9.9
34variably protease-sensitive prionopathy9.9
35tonsillitis9.8
36autonomic neuropathy9.8
37alagille syndrome9.8MSMB, PRND, PRNP
38berylliosis9.8CAT, SOD2
39huntington disease9.7
40spinal cord injury9.7
41colitis9.7
42myiasis9.7
43diarrhea9.7
44lateral sclerosis9.7
45retinitis9.7
46cerebellar degeneration9.7
47spastic paraparesis9.7
48wallerian degeneration9.7
49familial creutzfeldt-jakob disease9.7
50ataxia9.7

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



Diseases related to prion disease

Symptoms for Prion Disease

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Drugs & Therapeutics for Prion Disease

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Drugs for Prion Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
QuinacrinePhase 2683-89-6237
Synonyms:
130-42-7
130-42-7 (mono-hydrochloride)
2-Methoxy-6-chloro-9-diethylaminopentylaminoacridine
3-Chloro-7-methoxy-9-(1-methyl-4-diethylaminobutylamino)acridine
3-chloro-7-Methoxy-9-(1-methyl-4-diethylaminobutylamino)acridine
316-05-2 (dimesylate)
4-N-(6-chloro-2-methoxyacridin-9-yl)-1-N,1-N-diethylpentane-1,4-diamine
6-Chloro-9-((4-(diethylamino)-1-methylbutyl)amino)-2-methoxyacridine
6-chloro-9-((4-(Diethylamino)-1-methylbutyl)amino)-2-methoxyacridine
6151-30-0
6151-30-0 (di-hydrochloride, di-hydrate)
69-05-6
69-05-6 (di-hydrochloride)
78901-94-7
78901-94-7 (monoacetate)
83-89-6
83-89-6 (Parent)
866 R.P.
AB00053540
AC1L18SD
AC1L3ZIX
AC1MBFYF
AC1NS008
AC1Q2BPT
AI3-04467
AKOS000541535
AKOS004910405
Acirchine
Acrinamine
Acriquine
Akrichin
Antimalarina
Arichin
Atabrine dihydrochloride
Atabrine hydrochloride
Atabrine hydrochloride dihydrate
Atebrine
BAS 00439963
BPBio1_000348
BRD-A45889380-336-03-4
BSPBio_000316
BSPBio_002112
C07339
C23H30ClN3O.2HCl.2H2O
CCRIS 8633
CHEBI:8711
CHEMBL546257
CHEMBL7568
CID149640
CID237
CID2725002
CID5351141
CPD000449289
D08179
DB01103
DivK1c_000101
EINECS 201-508-7
EINECS 204-989-1
Erion
Erion Hydrochloride
GNF-PF-5448
HMS1568P18
HMS2090L03
HSDB 3253
Haffkinine
IDI1_000101
Italchin
Italchine
KBio1_000101
KBio2_001409
KBio2_002507
KBio2_003977
KBio2_005075
KBio2_006545
KBio2_007643
KBio3_001612
KBio3_002985
KBioGR_001011
KBioGR_002507
 
KBioSS_001409
KBioSS_002515
LS-14293
LS-14294
LS-190025
Lopac0_000970
MLS000758289
MS-1557
Malaricida
Mecryl
Mepacrina
Mepacrina [INN-Spanish]
Mepacrine
Mepacrine (INN)
Mepacrine HCl
Mepacrine [INN:BAN]
Mepacrine hydrochloride dihydrate
Mepacrinum
Mepacrinum [INN-Latin]
Methoquine
Metochin
MolPort-001-739-072
MolPort-004-285-521
N'-(6-chloro-2-methoxyacridin-9-yl)-N,N-diethylpentane-1,4-diamine
N(4)-(6-chloro-2-Methoxy-9-acridinyl)-N(1),N(1)-diethyl-1,4-pentanediamine
N(4)-(6-chloro-2-methoxy-9-acridinyl)-N(1),N(1)-diethyl-1,4-pentanediamine
N*4*-(6-Chloro-2-methoxy-acridin-9-yl)-N*1*,N*1*-diethyl-pentane-1,4-diamine
N4-(6-Chloro-2-methoxy-9-acridinyl)-N1,N1-diethyl-1,4-pentanediamine
N4-(6-Chloro-2-methoxy-9-acridinyl)-N1,N1-diethyl-1,4-pentanediamine monoacetate
NCGC00015874-04
NCGC00162300-01
NCGC00180899-01
NCGC00180969-01
NCI60_000926
NINDS_000101
NSC14229
N~4~-(6-chloro-2-methoxyacridin-9-yl)-N~1~,N~1~-diethylpentane-1,4-diamine
N~4~-[6-chloro-2-(methyloxy)acridin-9-yl]-N~1~,N~1~-diethylpentane-1,4-diamine
Palacrin
Palusan
Pentilen
Prestwick0_000318
Prestwick1_000318
Prestwick2_000318
Prestwick3_000318
Prestwick_650
Quinacrin
Quinacrine
Quinacrine Dihydrochloride
Quinacrine Dihydrochloride, Dihydrate
Quinacrine acetate
Quinacrine dihydrochloride dihydrate
Quinacrine dihydrochloride mustard
Quinacrine hydrochloride dihydrate
Quinactine
RP 866
RP-866 (Dihydrochloride dihydrate)
SAM001247040
SMR000449289
SN-390 (Dihydrochloride dihydrate)
SPBio_000676
SPBio_002535
STL061087
Spectrum2_000888
Spectrum3_000606
Spectrum4_000496
Spectrum5_001405
Spectrum_000929
St 439
TCMDC-125480
WLN: T C666 BNJ EG IMY1&3N2&2 LO1 &GH 2
acetic acid
acrichine
atabrine
atebrin
cMAP_000067
chinacrin
mepacrine
nchembio.215-comp6
nchembio873-comp30
quinacrine
2THROMBIN284

Interventional clinical trials:

idNameStatusNCT IDPhase
1Vasculopathic Injury and Plasma as Endothelial Rescue - OCTAplas Trial (EudraCT no. 2014-000452-28)Active, not recruitingNCT02253082Phase 4
2Safety and Efficacy Study to Compare Uniplas With Cryosupernatant Plasma in Thrombotic Thrombocytopenic Purpura (TTP)TerminatedNCT00411801Phase 3
3CJD (Creutzfeldt-Jakob Disease) Quinacrine StudyCompletedNCT00183092Phase 2
4Study of Ruxolitinib in the Treatment of Cachexia in Patients With Tumor-Associated Chronic Wasting Diseases.Active, not recruitingNCT02072057Phase 2
5PRION-1: Quinacrine for Human Prion DiseaseCompletedNCT00104663
6The Molecular Biology of Paroxysmal Nocturnal Hemoglobinuria (PNH)CompletedNCT00721864
7Therapeutic Antibodies Against Prion Diseases From PRNP Mutation CarriersRecruitingNCT02837705
8Enhanced CJD Surveillance in the Older PopulationRecruitingNCT02629640
9The Role of the Coagulation Pathway at the Synapse in Prion DiseasesNot yet recruitingNCT02480725
10Notification of Donors With Positive Microbiology MarkersNot yet recruitingNCT01050881

Search NIH Clinical Center for Prion Disease


Cochrane evidence based reviews: prion diseases

Genetic Tests for Prion Disease

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Anatomical Context for Prion Disease

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MalaCards organs/tissues related to Prion Disease:

34
Brain, Cortex, Endothelial, Testes, Spleen, Tonsil, Bone

Animal Models for Prion Disease or affiliated genes

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MGI Mouse Phenotypes related to Prion Disease:

39
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053877.8ADAM10, CD40, CR2, IL1B, MSMB, PRND
2MP:00053977.6ADAM10, CD40, CR2, IL1B, PRNP, SCN2A
3MP:00107686.7ADAM10, CAT, CR2, IL1B, MAP2, PRNP
4MP:00053766.3ADAM10, CAT, CD40, CR2, IL1B, LDLRAP1
5MP:00036316.3ADAM10, CD40, ENO2, IL1B, MAP2, PRND

Publications for Prion Disease

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Articles related to Prion Disease:

(show top 50)    (show all 616)
idTitleAuthorsYear
1
Increases of Galectin-1 and its S-nitrosylated form in the Brain Tissues of Scrapie-Infected Rodent Models and Human Prion Diseases. (27211330)
2016
2
Naturally occurring genetic variation that prevents prion disease is identified. (26071523)
2015
3
Parkinson's disease and prion disease: Straining the comparison. (26769458)
2015
4
Prion Disease Induces Alzheimer Disease-Like Neuropathologic Changes. (26226132)
2015
5
Profoundly different prion diseases in knock-in mice carrying single PrP codon substitutions associated with human diseases. (23959875)
2013
6
A novel familial prion disease causing pan-autonomic-sensory neuropathy and cognitive impairment. (23577609)
2013
7
Levels of the Mahogunin Ring Finger 1 E3 ubiquitin ligase do not influence prion disease. (23383230)
2013
8
Brain region specific pre-synaptic and post-synaptic degeneration are early components of neuropathology in prion disease. (23383030)
2013
9
2-aminothiazoles with improved pharmacotherapeutic properties for treatment of prion disease. (23509039)
2013
10
Human prion diseases: from kuru to variant creutzfeldt-jakob disease. (23225013)
2012
11
Remarkable reduction of MAP2 in the brains of scrapie-infected rodents and human prion disease possibly correlated with the increase of calpain. (22272295)
2012
12
Prolongation of prion disease-associated symptomatic phase relates to CD3+ T cell recruitment into the CNS in murine scrapie-infected mice. (22522067)
2012
13
Structure-activity relationship refinement and further assessment of indole-3-glyoxylamides as a lead series against prion disease. (21154498)
2011
14
Genome wide association studies and prion disease. (21844666)
2011
15
Rescuing neurons in prion disease. (21158739)
2011
16
Familial prion disease with Alzheimer disease-like tau pathology and clinical phenotype. (21416485)
2011
17
Prion disease susceptibility is affected by beta-structure folding propensity and local side-chain interactions in PrP. (21041683)
2010
18
Review: contribution of transgenic models to understanding human prion disease. (20880036)
2010
19
HECTD2 is associated with susceptibility to mouse and human prion disease. (19214206)
2009
20
Therapeutic interventions ameliorating prion disease. (19622059)
2009
21
Shadoo (Sprn) and prion disease incubation time in mice. (19513788)
2009
22
Impaired axonal transport in motor neurons correlates with clinical prion disease. (19696919)
2009
23
Unaltered SNARE complex formation in an in vivo model of prion disease. (18706894)
2008
24
A manganese-superoxide dismutase/catalase mimetic extends survival in a mouse model of human prion disease. (18455516)
2008
25
Synthesis and evaluation of a focused library of pyridine dicarbonitriles against prion disease. (17475368)
2008
26
Washington statewide pathology surveillance for prion disease. (17358023)
2007
27
Neuropathological diagnosis of prion disease]. (17695276)
2007
28
Surveillance for prion disease in cervids, Germany. (16494763)
2006
29
Human prion disease hypothesis does not justify the origin of bovine spongiform encephalopathy. (16855329)
2006
30
Involvement of the peripheral nervous system in human prion diseases including dural graft associated Creutzfeldt-Jakob disease. (15716520)
2005
31
Neurodegeneration and oxidative stress: prion disease results from loss of antioxidant defence. (16416388)
2005
32
Chronic wasting disease--prion disease in the wild. (15094817)
2004
33
Prion disease and Alzheimer's disease: pathogenic overlap. (15190676)
2004
34
Phenotypic variability in the brains of a family with a prion disease characterized by a 144-base pair insertion in the prion protein gene. (12662318)
2003
35
Academy calls for improved tests to beat prion disease. (14628008)
2003
36
A murine model of a familial prion disease. (12733431)
2003
37
Prion protein interactions with nucleic acid: possible models for prion disease and prion function. (12718450)
2003
38
Prion disease: bridging the spleen-nerve gap. (14647521)
2003
39
Copper binding to PrPC may inhibit prion disease propagation. (14642846)
2003
40
Soluble dimeric prion protein binds PrP(Sc) in vivo and antagonizes prion disease. (12679034)
2003
41
Is there a risk of prion disease after the administration of urinary-derived gonadotrophins? (12093821)
2002
42
Mayhem of the multiple mechanisms: modelling neurodegeneration in prion disease. (12124421)
2002
43
Prion disease: close encounters of the cellular kind. (11937041)
2002
44
Identification of multiple quantitative trait loci linked to prion disease incubation period in mice. (11353827)
2001
45
Aberrant metal binding by prion protein in human prion disease. (11579148)
2001
46
Alzheimer's and prion disease as disorders of protein conformation: implications for the design of novel therapeutic approaches. (10426190)
1999
47
An inherited prion disease with a PrP P105L mutation: clinicopathologic and PrP heterogeneity. (10408557)
1999
48
Effectiveness of anthracycline against experimental prion disease in Syrian hamsters. (9148807)
1997
49
Prion diseases and a new variant of Creutzfeldt-Jakob disease]. (9128415)
1996
50
Prion protein transgenes and the neuropathology in prion diseases. (7767493)
1995

Variations for Prion Disease

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Expression for genes affiliated with Prion Disease

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Search GEO for disease gene expression data for Prion Disease.

Pathways for genes affiliated with Prion Disease

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Pathways related to Prion Disease according to KEGG:

32
id Name KEGG Source Accession
1Prion diseaseshsa05020

Pathways related to Prion Disease according to GeneCards Suite gene sharing:

idSuper pathwaysScoreTop Affiliating Genes
19.6IL1B, MAP2, TF
29.1CAT, SOD2
39.0ENO2, MAP2, PRNP, SCN2A
4
Show member pathways
8.9CAT, IL1B, SOD2

GO Terms for genes affiliated with Prion Disease

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Cellular components related to Prion Disease according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1basal plasma membraneGO:000992510.2LDLRAP1, TF
2anchored component of membraneGO:00312259.8PRND, PRNP, SPRN
3vesicleGO:00319829.8IL1B, SPRN, TF
4extracellular spaceGO:00056157.8CAT, CD40, ENO2, IL1B, MSMB, TF
5extracellular exosomeGO:00700626.8ADAM10, CAT, CD40, CR2, ENO2, IL1B

Biological processes related to Prion Disease according to GeneCards Suite gene sharing:

(show all 13)
idNameGO IDScoreTop Affiliating Genes
1cellular copper ion homeostasisGO:000687810.4PRND, PRNP
2B cell proliferationGO:004210010.1CD40, CR2
3positive regulation of receptor-mediated endocytosisGO:004826010.0LDLRAP1, TF
4response to ozoneGO:001019310.0CAT, IL1B
5iron ion homeostasisGO:00550729.9SOD2, TF
6response to gamma radiationGO:00103329.7IL1B, SOD2
7response to hyperoxiaGO:00550939.4CAT, SOD2
8positive regulation of NF-kappaB transcription factor activityGO:00510929.4CAT, CD40, IL1B
9response to L-ascorbic acidGO:00335919.4CAT, IL1B, SOD2
10response to cadmium ionGO:00466869.2CAT, PRNP, SOD2
11response to oxidative stressGO:00069799.0CAT, PRNP, SOD2
12response to hypoxiaGO:00016668.9CAT, IL1B, SOD2
13protein homooligomerizationGO:00512608.8PRND, PRNP, SOD2

Sources for Prion Disease

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet