MCID: PRN023
MIFTS: 42

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 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

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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 personality disorder. 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 endothelial, 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

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

(show top 50)    (show all 430)
idRelated DiseaseScoreTop Affiliating Genes
1prion disease with protracted course33.3APP, PRNP, YWHAZ
2personality disorder29.8APP, SPRN
3atrial fibrillation29.7MAPT, PRNP, YWHAZ
4atopic dermatitis29.2APP, IL1B, MAPT, PRNP
5gerstmann-straussler disease29.2APP, MAPT, PRND, PRNP, RPSA, SPRN
6genetic prion diseases12.6
7familial alzheimer-like prion disease12.3
8huntington disease-like 111.3
9creutzfeldt-jakob disease10.9
10insomnia, fatal familial10.5
11variably protease-sensitive prionopathy10.4
12localized pagetoid reticulosis10.3PRND, PRNP
13leukemia10.3
14lymphoma10.3
15kuru10.2
16huriez syndrome10.2
17breast cancer10.2
18mucinous cystadenocarcinoma10.2CAT, MAP2, PRNP
19amyotrophic lateral sclerosis 710.2PRNP, YWHAZ
20melanoma10.2
21malignant growth hormone secreting neoplasm of pituitary10.2CD40, CR2
22mitochondrial metabolism disease10.2IL1B, MAPT, PRNP
23obesity10.1
24esophagitis10.1
25cerebritis10.1
26pancreatitis10.1
27thyroiditis10.1
28malaria10.1
29hepatitis10.1
30prostatitis10.1
31cervicitis10.1
32adenocarcinoma10.1
33adenoma10.1
34herpes simplex10.1
35endotheliitis10.1
36familial mediterranean fever type 110.1MSMB, PRND, PRNP
37harp syndrome10.1PRND, PRNP, YWHAZ
38basophil adenoma10.1MAPT, YWHAZ
39encephalitozoonosis10.0MAPT, PRNP, YWHAZ
40myocardial infarction10.0
41systemic lupus erythematosus10.0
42lung cancer10.0
43hepatocellular carcinoma10.0
44coronary artery disease10.0
45cystic fibrosis10.0
46acute myocardial infarction10.0
47b-cell lymphomas10.0
48artery disease10.0
49urethritis10.0
50gingivitis10.0

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):

(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

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Anatomical Context for Prion Disease

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MalaCards organs/tissues related to Prion Disease:

33
Brain, Cortex, Endothelial, Breast, T cells, Lung, Kidney

Animal Models for Prion Disease or affiliated genes

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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

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Articles related to Prion Disease:

(show top 50)    (show all 592)
idTitleAuthorsYear
1
Brain-derived neurotrophic factor serum levels in genetically isolated populations: gender-specific association with anxiety disorder subtypes but not with anxiety levels or Val66Met polymorphism. (26539329)
2015
2
Nonhepatic hyperammonemic encephalopathy due to undiagnosed urea cycle disorder. (26130895)
2015
3
Improved mortality rate for congenital diaphragmatic hernia in the modern era of management: 15year experience in a single institution. (25840055)
2015
4
Phacomatosis pigmentovascularis. (26513082)
2015
5
Role of CXCL10 in cryoglobulinemia. (25571936)
2015
6
Proteasome-dependent degradation of transcription factor activating enhancer-binding protein 4 (TFAP4) controls mitotic division. (24500709)
2014
7
Cell fate factor DACH1 represses YB-1-mediated oncogenic transcription and translation. (24335958)
2014
8
Brk/PTK6 cooperates with HER2 and Src in regulating breast cancer cell survival and epithelial-to-mesenchymal transition. (23291984)
2013
9
Use of cyclosporine A and tacrolimus in treatment of vernal keratoconjunctivitis. (23625179)
2013
10
Ivabradine in treatment of sinus tachycardia mediated vasovagal syncope. (24072450)
2013
11
Double-barrel right ventricular outflow: tetralogy of Fallot annulus preservation technique. (22916765)
2012
12
Head injury or head motion? Assessment and quantification of motion artifacts in diffusion tensor imaging studies. (21391258)
2012
13
Evaluation of total and corneal wavefront high order aberrations for the detection of forme fruste keratoconus. (22427590)
2012
14
Linagliptin, a dipeptidyl peptidase-4 inhibitor with a unique pharmacological profile, and efficacy in a broad range of patients with type 2 diabetes. (22149370)
2012
15
Systematic evaluation of genetic variants in three biological pathways on patient survival in low-stage non-small cell lung cancer. (21792076)
2011
16
Complete resolution of emphysematous gastritis after conservative management. (21070878)
2011
17
Neurodevelopmental and neurofunctional outcomes in children with congenital diaphragmatic hernia. (21640525)
2011
18
Cochlear implantation in a patient with superficial siderosis: an update. (20015783)
2010
19
Acute lymphoblastic leukemia masquerading as juvenile rheumatoid arthritis: diagnostic pitfall and association with survival. (19727722)
2010
20
The role for BMT in erythropoietic protoporphyria. (19525986)
2010
21
The expression of hepatocyte growth factor and heparanase in laryngeal squamous cell carcinoma and its clinical significance]. (19452723)
2009
22
Entero-insular axis in children with simple obesity. (19772813)
2009
23
Juvenile-onset G(M2)-gangliosidosis in an African-American child with nystagmus. (18358410)
2008
24
GPCR-jacking: from a new route in RTK signalling to a new concept in GPCR activation. (18001849)
2007
25
A genetic association analysis of cognitive ability and cognitive ageing using 325 markers for 109 genes associated with oxidative stress or cognition. (17601350)
2007
26
Peritoneal tuberculosis mimicking advanced ovarian cancer: a retrospective review of 22 cases. (16740297)
2006
27
The effect of calcium channel blocker lercanidipine on lowgrade inflammation parameters in essential hypertension patients]. (17220028)
2006
28
Interleukin-10 - 1082 GG polymorphism influences the occurrence and the clinical characteristics of hepatitis C virus infection. (17049666)
2006
29
Osteopontin in rheumatoid arthritis]. (15799343)
2005
30
The epigenetic breakdown of cancer cells: from DNA methylation to histone modifications. (15881895)
2005
31
The modulating of aloe polysaccharides on the cell cycle and cycle regulating protein expression in X-ray irradiated non-malignant cells]. (16209264)
2005
32
Recent advances on the role of CD40 and dendritic cells in immunity and tolerance. (12799532)
2003
33
Serum squamous cell carcinoma antigen is a useful biologic marker in patients with inverted papillomas of the sinonasal tract. (11815971)
2002
34
Familial dilated cardiomyopathy. (12621922)
2002
35
High or low hematocrits during cardiopulmonary bypass for patients undergoing coronary artery bypass graft surgery? An evidence-based approach to the question. (11958311)
2002
36
Mutation and expression of the beta-catenin-interacting protein ICAT in human colorectal tumors. (12417602)
2002
37
Role of the ribosomal stalk components in the resistance of Aspergillus fumigatus to the sordarin antifungals. (11849550)
2002
38
Heparanase protein and gene expression in bladder cancer. (11547059)
2001
39
The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals. (11226231)
2001
40
Combination therapy with insulin and metformin. (15251717)
1998
41
A prospective, randomized, double-blind multicenter trial of a single bolus injection of the novel modified t-PA E6010 in the treatment of acute myocardial infarction: comparison with native t-PA. E6010 Study Group. (9180103)
1997
42
Transgene expression of plasmid DNAs directed by viral or neural promoters in the rat brain. (9191082)
1997
43
New non-sulfonylurea insulin secretagogues. (15989662)
1997
44
The assessment and nutritional support of patients with the acquired immunodeficiency syndrome]. (7711150)
1995
45
Acyclovir for childhood chickenpox. Cost is unjustified. (7833700)
1995
46
Combined use of serum enzyme levels as tumor markers in cervical carcinoma patients. (8146529)
1994
47
The Klippel-Trenaunay syndrome. (1846865)
1991
48
Modulation of growth and differentiation in normal human keratinocytes by transforming growth factor-beta. (2211846)
1990
49
50

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:

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

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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

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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