MCID: PRN023
MIFTS: 50

Prion Disease malady

Categories: Neuronal diseases, Mental diseases

Aliases & Classifications for Prion Disease

About this section

Aliases & Descriptions for Prion Disease:

Name: Prion Disease 10 68 23 12
Prion Diseases 46 47 36 2 65
Prion Disease Pathway 10 65
Human Transmissible Spongiform Encephalopathies, Inherited 65
Inherited Human Transmissible Spongiform Encephalopathies 23
Transmissible Spongiform Encephalopathies 23
Transmissible Spongiform Encephalopathy 10
Prion-Associated Disorders 23
 
Spongiform Encephalopathy 10
Transmissible Dementias 23
Prion-Induced Disorders 23
Prion Protein Diseases 23
Prion Induced Disorder 10
Prion Protein Disease 10
Prion Protein 11
Tses 23

Classifications:



External Ids:

Disease Ontology10 DOID:649
ICD1027 A81.9
MeSH36 D017096
NCIt42 C27585
UMLS65 C0162534, C3536911, C0751645

Summaries for Prion Disease

About this section
Genetics Home Reference:23 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 prion disease with protracted course and gerstmann-straussler disease. An important gene associated with Prion Disease is PRNP (Prion Protein), and among its related pathways are Glypican 1 network and A-beta Pathways: Plaque Formation and APP Metabolism. Affiliated tissues include brain, cortex and b cells, and related mouse phenotypes are tumorigenesis and endocrine/exocrine gland.

Disease Ontology:10 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:46 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:68 Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of... more...

Related Diseases for Prion Disease

About this section

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

(show top 50)    (show all 62)
idRelated DiseaseScoreTop Affiliating Genes
1prion disease with protracted course32.5APP, PRNP, YWHAZ
2gerstmann-straussler disease27.7APP, MAPT, PRND, PRNP, RPSA, SPRN
3genetic prion diseases12.3
4familial alzheimer-like prion disease11.9
5huntington disease-like 110.9
6creutzfeldt-jakob disease10.8
7kuru10.6
8localized pagetoid reticulosis10.5PRND, PRNP
9mucinous cystadenocarcinoma10.3CAT, MAP2, PRNP
10scrapie10.3
11encephalopathy10.3
12amyotrophic lateral sclerosis 710.3PRNP, YWHAZ
13neuronitis10.3
14malignant growth hormone secreting neoplasm of pituitary10.3CD40, CR2
15mitochondrial metabolism disease10.2IL1B, MAPT, PRNP
16chronic wasting disease10.2
17insomnia, fatal familial10.2
18familial mediterranean fever type 110.1MSMB, PRND, PRNP
19harp syndrome10.1PRND, PRNP, YWHAZ
20basophil adenoma10.1MAPT, YWHAZ
21atrial fibrillation10.0MAPT, PRNP, YWHAZ
22encephalitozoonosis10.0MAPT, PRNP, YWHAZ
23dementia10.0
24unverricht-lundborg syndrome10.0CR2, PRNP, RPSA, SPRN
25cerebritis10.0
26variably protease-sensitive prionopathy10.0
27alzheimer disease9.9
28neuropathy9.9
29retinoblastoma fa - friedreich ataxia9.9APP, MAPT
30personality disorder9.9APP, SPRN
31spermatogenic failure 109.9APP, MAPT
32huriez syndrome9.9
33birdshot chorioretinopathy9.8APP, MAPT
34tonsillitis9.8
35autonomic neuropathy9.8
36milker's nodule9.7APP, MAPT, PRNP
37parkinson disease 19.7APP, MAPT, PRNP
38mast syndrome9.7APP, MAPT
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

About this section

Drugs & Therapeutics for Prion Disease

About this section

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

(show all 9)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1Antigen-Antibody ComplexPhase 47
2
QuinacrineapprovedPhase 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
3Antiprotozoal AgentsPhase 21730
4Coal TarPhase 2165
5Antiparasitic AgentsPhase 21854
6AnthelminticsPhase 2379
7Anti-Infective AgentsPhase 217220
8AntimalarialsPhase 21058
9Thrombin269

Interventional clinical trials:

idNameStatusNCT IDPhase
1Vasculopathic Injury and Plasma as Endothelial Rescue - OCTAplas Trial (EudraCT no. 2014-000452-28)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
7Enhanced CJD Surveillance in the Older PopulationRecruitingNCT02629640
8The Role of the Coagulation Pathway at the Synapse in Prion DiseasesNot yet recruitingNCT02480725
9Notification 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

About this section

Anatomical Context for Prion Disease

About this section

MalaCards organs/tissues related to Prion Disease:

33
Brain, Cortex, B cells, Pituitary, Endothelial, Breast, Heart

Animal Models for Prion Disease or affiliated genes

About this section

MGI Mouse Phenotypes related to Prion Disease:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.8CAT, FPR2, IL1B, MAPT, MSMB
2MP:00053797.9ADAM10, CD40, FPR2, MAP2, MSMB, PRNP
3MP:00036317.3ADAM10, APP, CD40, IL1B, MAP2, MAPT
4MP:00107686.6ADAM10, APP, CAT, CR2, FPR2, IL1B
5MP:00053976.3ADAM10, APP, CD40, CR2, FPR2, IL1B
6MP:00053875.7ADAM10, APP, CD40, CR2, FPR2, IL1B

Publications for Prion Disease

About this section

Articles related to Prion Disease:

(show top 50)    (show all 613)
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
Clinically Unsuspected Prion Disease Among Patients With Dementia Diagnoses in an Alzheimer's Disease Database. (26320231)
2015
3
Methylphenidate prolongs symptom-free period of experimental prion disease in mice. (25748967)
2015
4
Naturally occurring genetic variation that prevents prion disease is identified. (26071523)
2015
5
Parkinson's disease and prion disease: Straining the comparison. (26769458)
2015
6
Prion Disease Induces Alzheimer Disease-Like Neuropathologic Changes. (26226132)
2015
7
Descriptive Epidemiology of Prion Disease in Japan: 1999-2012. (25283311)
2014
8
Profoundly different prion diseases in knock-in mice carrying single PrP codon substitutions associated with human diseases. (23959875)
2013
9
A novel familial prion disease causing pan-autonomic-sensory neuropathy and cognitive impairment. (23577609)
2013
10
Levels of the Mahogunin Ring Finger 1 E3 ubiquitin ligase do not influence prion disease. (23383230)
2013
11
Human prion diseases: from kuru to variant creutzfeldt-jakob disease. (23225013)
2012
12
Remarkable reduction of MAP2 in the brains of scrapie-infected rodents and human prion disease possibly correlated with the increase of calpain. (22272295)
2012
13
Change in the characteristics of ferritin induces iron imbalance in prion disease affected brains. (22182691)
2012
14
Molecular diagnosis of human prion disease. (23484550)
2011
15
From high-throughput cell culture screening to mouse model: identification of new inhibitor classes against prion disease. (21755599)
2011
16
Structure-activity relationship refinement and further assessment of indole-3-glyoxylamides as a lead series against prion disease. (21154498)
2011
17
The role of predation in disease control: a comparison of selective and nonselective removal on prion disease dynamics in deer. (21269999)
2011
18
Gene knockout of tau expression does not contribute to the pathogenesis of prion disease. (22002429)
2011
19
All clinically-relevant blood components transmit prion disease following a single blood transfusion: a sheep model of vCJD. (21858015)
2011
20
Can prion disease suspicion be supported earlier? Clinical, radiological and laboratory findings in a series of cases. (21869605)
2011
21
Residues surrounding the glycosylphosphatidylinositol anchor attachment site of PrP modulate prion infection: insight from the resistance of rabbits to prion disease. (20427543)
2010
22
Prion disease susceptibility is affected by beta-structure folding propensity and local side-chain interactions in PrP. (21041683)
2010
23
Pathologic evidence that the T188R mutation in PRNP is associated with prion disease. (21107135)
2010
24
Change in tau phosphorylation associated with neurodegeneration in the ME7 model of prion disease. (20298219)
2010
25
A Copine family member, Cpne8, is a candidate quantitative trait gene for prion disease incubation time in mouse. (19795140)
2010
26
Reduction of prion infectivity and levels of scrapie prion protein by lithium aluminum hydride: implications for RNA in prion diseases. (19606066)
2009
27
HECTD2 is associated with susceptibility to mouse and human prion disease. (19214206)
2009
28
Unaltered SNARE complex formation in an in vivo model of prion disease. (18706894)
2008
29
Mutant prion protein D202N associated with familial prion disease is retained in the endoplasmic reticulum and forms 'curly' intracellular aggregates. (17873292)
2007
30
Kuru in the 21st century--an acquired human prion disease with very long incubation periods. (16798390)
2006
31
Regional brain metabolite abnormalities in inherited prion disease and asymptomatic gene carriers demonstrated in vivo by quantitative proton magnetic resonance spectroscopy. (16598479)
2006
32
Incubation period of human prion disease. (16962868)
2006
33
Redox metals and oxidative abnormalities in human prion diseases. (16096758)
2005
34
Involvement of the peripheral nervous system in human prion diseases including dural graft associated Creutzfeldt-Jakob disease. (15716520)
2005
35
Neurodegeneration and oxidative stress: prion disease results from loss of antioxidant defence. (16416388)
2005
36
Chronic wasting disease--prion disease in the wild. (15094817)
2004
37
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
38
Academy calls for improved tests to beat prion disease. (14628008)
2003
39
Prion diseases. (12707849)
2003
40
A murine model of a familial prion disease. (12733431)
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
Identification of multiple quantitative trait loci linked to prion disease incubation period in mice. (11353827)
2001
44
Transgenic models of prion disease. (11214913)
2000
45
Inherited prion disease with an alanine to valine mutation at codon 117 in the prion protein gene. (10506086)
1999
46
Retrospective neuropathological review of prion disease in UK haemophilic patients. (9869159)
1998
47
Effectiveness of anthracycline against experimental prion disease in Syrian hamsters. (9148807)
1997
48
Molecular genetics of human prion diseases. (7913754)
1994
49
50

Variations for Prion Disease

About this section

Expression for genes affiliated with Prion Disease

About this section
Search GEO for disease gene expression data for Prion Disease.

Pathways for genes affiliated with Prion Disease

About this section

Pathways related to Prion Disease according to KEGG:

31
id Name KEGG Source Accession
1Prion diseaseshsa05020

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

idSuper pathwaysScoreTop Affiliating Genes
19.7APP, PRNP
29.7ADAM10, APP
39.3ADAM10, APP, MAPT
49.3IL1B, MAP2, YWHAZ
5
Show member pathways
9.0ADAM10, APP, IL1B, MAPT
69.0CD40, IL1B, RPSA
78.7APP, CD40, FPR2
8
Show member pathways
8.4CD40, IL1B, RPSA, YWHAZ

GO Terms for genes affiliated with Prion Disease

About this section

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

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1response to L-ascorbic acidGO:003359110.1CAT, IL1B
2response to cadmium ionGO:004668610.1CAT, PRNP
3response to ozoneGO:00101939.9CAT, IL1B
4cholesterol metabolic processGO:00082039.8APP, CAT
5purine nucleobase metabolic processGO:00061449.8CAT, IL1B
6response to oxidative stressGO:00069799.2APP, CAT, PRNP
7platelet activationGO:00301688.2APP, CD40, YWHAZ

Sources for Prion Disease

About this section
2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet