TSES
MCID: PRN023
MIFTS: 48

Prion Disease (TSES) malady

Categories: Neuronal diseases, Mental diseases

Aliases & Classifications for Prion Disease

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Aliases & Descriptions for Prion Disease:

Name: Prion Disease 11 25 13
Prion Diseases 49 50 2 39 68
Prion Disease Pathway 11 68
Human Transmissible Spongiform Encephalopathies, Inherited 68
Inherited Human Transmissible Spongiform Encephalopathies 25
Transmissible Spongiform Encephalopathies 25
Transmissible Spongiform Encephalopathy 11
Prion-Associated Disorders 25
 
Spongiform Encephalopathy 11
Transmissible Dementias 25
Prion-Induced Disorders 25
Prion Induced Disorder 11
Prion Protein Diseases 25
Prion Protein Disease 11
Prion Protein 12
Tses 25

Classifications:



External Ids:

Disease Ontology11 DOID:649
ICD1030 A81.9
MeSH39 D017096
NCIt45 C27585

Summaries for Prion Disease

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Genetics Home Reference:25 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 huntington disease-like 1. An important gene associated with Prion Disease is PRNP (Prion Protein), and among its related pathways are Glypican 1 network and Taxane Pathway, Pharmacokinetics. Affiliated tissues include brain, cortex and testes, and related mouse phenotypes are neoplasm and cellular.

CDC:2 Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.

NINDS:49 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.

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.

Wikipedia:71 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 57)
idRelated DiseaseScoreTop Affiliating Genes
1prion disease with protracted course32.1MAPT, MSMB, PRND, PRNP, RPSA, SPRN
2huntington disease-like 131.3APP, MSMB, PRNP
3genetic prion diseases12.3
4familial alzheimer-like prion disease11.8
5creutzfeldt-jakob disease11.4
6gerstmann-straussler disease11.1
7kuru11.1
8insomnia, fatal familial11.0
9huriez syndrome10.7
10variably protease-sensitive prionopathy10.7
11scrapie10.2
12charcot-marie-tooth disease10.2APP, IL1B, PRNP
13encephalopathy10.2
14adult choroid plexus cancer10.2CR2, PRNP, SPRN
15scrotum basal cell carcinoma10.2MAPT, PRNP
16ovarian mucinous adenocarcinoma10.2IL1B, MAP2, PRNP
17amelogenesis imperfecta, type iia310.2ADAM10, APP
18neuronitis10.1
19cervix disease10.1IL1B, MAP2, MAPT
20contagious pustular dermatitis10.1APP, MAPT, PRNP
21deafness, autosomal recessive 10110.1MAPT, PRNP
22coenzyme q10 deficiency, primary, 110.1APP, MAPT, PRNP
23fancf-related fanconi anemia10.1MSMB, PRND, PRNP
24alagille syndrome10.1MSMB, PRND, PRNP
25chronic wasting disease10.0
26hypoproteinemia, hypercatabolic10.0APP, MAPT, SOD2
27substance-induced psychosis10.0APP, IL1B, MAPT, PRNP
28epithelioid type angiomyolipoma10.0APP, IL1B, MAPT, PRNP
29tmem216-related meckel syndrome10.0TF, TFRC
30poems syndrome10.0TF, TFRC
31cat-scratch disease10.0CAT, TF, TFRC
32dementia10.0
33cerebritis9.9
34neuropathy9.9
35alzheimer disease9.8
36neuronal ceroid-lipofuscinoses9.7CAT, TF, TFRC
37tonsillitis9.7
38autonomic neuropathy9.7
39diarrhea9.7
40ataxia9.7
41long qt syndrome 59.7APP, MAP2, MAPT, PRNP, SOD2, TFRC
42spondyloarthropathy 19.7ADAM10, APP, CAT, IL1B, MAP2, MAPT
43cerebellar ataxia9.6
44huntington disease9.6
45spinal cord injury9.6
46colitis9.6
47myiasis9.6
48lateral sclerosis9.6
49retinitis9.6
50myopathy9.6

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



Diseases related to prion disease

Symptoms & Phenotypes for Prion Disease

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MGI Mouse Phenotypes related to Prion Disease according to GeneCards Suite gene sharing:

41
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.7CAT, FPR2, IL1B, MAPT, MSMB, SOD2
2MP:00053847.3ADAM10, APP, CAT, FPR2, MAPT, PRND
3MP:00053787.2ADAM10, APP, MAP2, MAPT, PRNP, RPSA
4MP:00053877.0ADAM10, APP, CR2, FPR2, IL1B, MAPT
5MP:00053976.7ADAM10, APP, CR2, FPR2, IL1B, MAPT
6MP:00036316.7ADAM10, APP, IL1B, MAP2, MAPT, PRND
7MP:00053766.6ADAM10, APP, CAT, CR2, FPR2, IL1B
8MP:00107685.5ADAM10, APP, CAT, CR2, FPR2, IL1B

Drugs & Therapeutics for Prion Disease

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

(show all 14)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1Antigen-Antibody ComplexPhase 4, Phase 210
2
QuinacrineapprovedPhase 2683-89-6237
Synonyms:
130-42-7
130-42-7 (mono-hydrochloride)
2-Methoxy-6-chloro-9-diethylaminopentylaminoacridine
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-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
N4-(6-chloro-2-methoxy-9-acridinyl)-N1,N1-diethyl-1,4-pentanediamine
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 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
3
Coal tarapprovedPhase 22028007-45-2
Synonyms:
 
Coal tars
Tar, coal
4AnthelminticsPhase 2446
5Anti-Infective AgentsPhase 222062
6Antiprotozoal AgentsPhase 22051
7Antiparasitic AgentsPhase 22199
8AntimalarialsPhase 21246
9Pharmaceutical SolutionsPhase 28192
10Antibodies6394
11Immunoglobulins6394
12Thrombin304
13Coagulants1500
14Hemostatics1443

Interventional clinical trials:

(show all 12)
idNameStatusNCT IDPhase
1Vasculopathic Injury and Plasma as Endothelial Rescue - OCTAplas Trial (EudraCT no. 2014-000452-28)CompletedNCT02253082Phase 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
4Vasculopathic Injury and Plasma as Endothelial Rescue in Septic Shock (SHOCK) TrialRecruitingNCT03092245Phase 2
5Study of Ruxolitinib in the Treatment of Cachexia in Patients With Tumor-Associated Chronic Wasting Diseases.Active, not recruitingNCT02072057Phase 2
6Notification of Donors With Positive Microbiology MarkersUnknown statusNCT01050881
7PRION-1: Quinacrine for Human Prion DiseaseCompletedNCT00104663
8The Molecular Biology of Paroxysmal Nocturnal Hemoglobinuria (PNH)CompletedNCT00721864
9Therapeutic Antibodies Against Prion Diseases From PRNP Mutation CarriersRecruitingNCT02837705
10Enhanced CJD Surveillance in the Older PopulationRecruitingNCT02629640
11Genetic Characterization of Movement Disorders and DementiasRecruitingNCT02014246
12The Role of the Coagulation Pathway at the Synapse in Prion DiseasesNot yet recruitingNCT02480725

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:

36
Brain, Cortex, Testes, Spleen, Tonsil, T cells, Bone

Publications for Prion Disease

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Articles related to Prion Disease:

(show top 50)    (show all 631)
idTitleAuthorsYear
1
Correction: Fatal Prion Disease in a Mouse Model of Genetic E200K Creutzfeldt-Jakob Disease. (28467504)
2017
2
Genetic Prion Disease Caused by PRNP Q160X Mutation Presenting with an Orbitofrontal Syndrome, Cyclic Diarrhea, and Peripheral Neuropathy. (27716661)
2017
3
Genetic prion disease: Experience of a rapidly progressive dementia center in the United States and a review of the literature. (27943639)
2017
4
Superficial bedside brain biopsy can be a safe and practical approach to confirm a rare form of prion disease in cerebellar ataxia: A case study. (28320192)
2017
5
Prion disease: experimental models and reality. (28084518)
2017
6
Neuroanatomical correlates of prion disease progression - a 3T longitudinal voxel-based morphometry study. (27942451)
2017
7
Increases of Galectin-1 and its S-nitrosylated form in the Brain Tissues of Scrapie-Infected Rodent Models and Human Prion Diseases. (27211330)
2016
8
Mononucleated Blood Cell Populations Display Different Abilities To Transmit Prion Disease by the Transfusion Route. (26764000)
2016
9
Transgenic mice recapitulate the phenotypic heterogeneity of genetic prion diseases without developing prion infectivity: Role of intracellular PrP retention in neurotoxicity. (26864450)
2016
10
Cannibalism, Kuru, and Mad Cows: Prion Disease As a "Choose-Your-Own-Experiment" Case Study to Simulate Scientific Inquiry in Large Lectures. (26788803)
2016
11
C-Terminal-Deleted Prion Protein Fragment Is a Major Accumulated Component of Systemic PrP Deposits in Hereditary Prion Disease With a 2-Bp (CT) Deletion in PRNP Codon 178. (27634965)
2016
12
Insights from Therapeutic Studies for PrP Prion Disease. (27836910)
2016
13
Structure-Based Drug Discovery for Prion Disease Using a Novel Binding Simulation. (27333028)
2016
14
Protective V127 prion variant prevents prion disease by interrupting the formation of dimer and fibril from molecular dynamics simulations. (26906032)
2016
15
The history of prion disease. (27751551)
2016
16
Diagnosis of Human Prion Disease Using Real-Time Quaking-Induced Conversion Testing of Olfactory Mucosa and Cerebrospinal Fluid Samples. (27942718)
2016
17
Correction: Cannibalism, Kuru, and Mad Cows: Prion Disease As a "Choose-Your-Own-Experiment" Case Study to Simulate Scientific Inquiry in Large Lectures. (26991940)
2016
18
Prion 2016 Animal Prion Disease Workshop Abstracts. (27088807)
2016
19
The real-time quaking-induced conversion assay for detection of human prion disease and study of other protein misfolding diseases. (27735933)
2016
20
Aggregation and Prion-Like Properties of Misfolded Tumor Suppressors: Is Cancer a Prion Disease? (27549118)
2016
21
The influence of commensal and pathogenic gut microbiota on prion disease pathogenesis. (27193137)
2016
22
Effect of Polylysine on Scrapie Prion Protein Propagation in Spleen during Asymptomatic Stage of Experimental Prion Disease in Mice. (27221113)
2016
23
'PrP systemic deposition disease': clinical and pathological characteristics of novel familial prion disease with 2-bp deletion in codon 178. (26768678)
2016
24
The stress of prion disease. (27060771)
2016
25
Positive 14-3-3 and tau proteins in a sporadic Creutzfeldt-Jakob disease case and a brief perspective of prion diseases in Colombia. (27622622)
2016
26
Increased Abundance of M Cells in the Gut Epithelium Dramatically Enhances Oral Prion Disease Susceptibility. (27973593)
2016
27
New blood tests make strides in detecting prion disease. (28008016)
2016
28
Over-Expressed Pathogenic miRNAs in Alzheimer's Disease (AD) and Prion Disease (PrD) Drive Deficits in TREM2-Mediated AI^42 Peptide Clearance. (27378912)
2016
29
MicroRNA abundance is altered in synaptoneurosomes during prion disease. (26658803)
2016
30
Increased circulating microRNAs miR-342-3p and miR-21-5p in natural sheep prion disease. (27959774)
2016
31
Chronic Progressive Neurodegeneration in a Transgenic Mouse Model of Prion Disease. (27891071)
2016
32
Quantifying prion disease penetrance using large population control cohorts. (26791950)
2016
33
Neil3 induced neurogenesis protects against prion disease during the clinical phase. (27886261)
2016
34
Clinically Unsuspected Prion Disease Among Patients With Dementia Diagnoses in an Alzheimer's Disease Database. (26320231)
2015
35
Genome-wide association study of behavioural and psychiatric features in human prion disease. (25897833)
2015
36
Proteinase-activated receptor 2 and disease biomarkers in cerebrospinal fluid in cases with autopsy-confirmed prion diseases and other neurodegenerative diseases. (25886404)
2015
37
Preclinical detection of infectivity and disease-specific PrP in blood throughout the incubation period of prion disease. (26631638)
2015
38
Shedding light on prion disease. (26186508)
2015
39
Inherited mtDNA variations are not strong risk factors in human prion disease. (26239179)
2015
40
Correction: PrPST, a Soluble, Protease Resistant and Truncated PrP Form Features in the Pathogenesis of a Genetic Prion Disease. (26193641)
2015
41
Methylphenidate prolongs symptom-free period of experimental prion disease in mice. (25748967)
2015
42
Genetics of Prion Disease in Cattle. (26462233)
2015
43
The Good, the Bad, and the Ugly of Dendritic Cells during Prion Disease. (26697507)
2015
44
The cognitive profile of prion disease: a prospective clinical and imaging study. (26000326)
2015
45
The Features of Genetic Prion Diseases Based on Chinese Surveillance Program. (26488179)
2015
46
Parkinson's disease and prion disease: Straining the comparison. (26769458)
2015
47
Expanding the prion disease repertoire. (26330608)
2015
48
Mutation protected mice in study against prion disease. (26390525)
2015
49
TDP-43 in amyotrophic lateral sclerosis - is it a prion disease? (25846565)
2015
50
Downregulation of I^-Soluble N-Ethylmaleimide-Sensitive Factor Attachment Protein: Proteomics-Based Identification in Early-Stage Prion Disease. (26022183)
2015

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:

34
id Name KEGG Source Accession
1Prion diseaseshsa05020

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

idSuper pathwaysScoreTop Affiliating Genes
19.8APP, PRNP
29.8MAP2, MAPT
39.8ADAM10, APP
49.4ADAM10, APP, MAPT
59.3TF, TFRC
69.2ADAM10, APP, MAPT, PRNP
79.2APP, MAP2, MAPT, PRNP
89.1CR2, IL1B, TFRC
99.1PRNP, TF, TFRC

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
1anchored component of membraneGO:003122510.6PRND, PRNP, SPRN
2nuclear peripheryGO:003439910.6MAP2, MAPT
3HFE-transferrin receptor complexGO:199071210.1TF, TFRC
4clathrin-coated pitGO:00059059.7APP, TF, TFRC
5cell surfaceGO:00099869.2ADAM10, APP, PRNP, TF, TFRC
6extracellular spaceGO:00056158.4ADAM10, APP, CAT, IL1B, MSMB, TF
7extracellular regionGO:00055768.2APP, CAT, IL1B, MSMB, PRND, SPRN
8extracellular exosomeGO:00700627.0ADAM10, APP, CAT, CR2, IL1B, PRNP
9plasma membraneGO:00058866.1ADAM10, APP, CAT, CR2, FPR2, MAPT

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

(show all 12)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of neuron deathGO:190121610.5MAPT, PRNP
2amyloid fibril formationGO:199000010.3APP, MAPT
3complement receptor mediated signaling pathwayGO:000243010.3CR2, FPR2
4cellular copper ion homeostasisGO:000687810.3APP, PRND, PRNP
5response to lead ionGO:001028810.1APP, CAT
6neuron projection developmentGO:003117510.0APP, MAP2, MAPT
7response to hyperoxiaGO:00550939.9CAT, SOD2
8response to L-ascorbic acidGO:00335919.9CAT, SOD2
9cellular response to drugGO:00356909.8IL1B, PRNP, TFRC
10response to cadmium ionGO:00466869.7CAT, PRNP, SOD2
11response to oxidative stressGO:00069799.2APP, CAT, PRNP, SOD2
12viral entry into host cellGO:00467188.9CR2, RPSA, TFRC

Molecular functions related to Prion Disease according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1complement receptor activityGO:000487510.4CR2, FPR2
2tubulin bindingGO:001563110.1MAP2, MAPT, PRNP
3enzyme bindingGO:00198999.3APP, CAT, MAPT, SOD2
4virus receptor activityGO:00016188.9CR2, RPSA, TFRC
5identical protein bindingGO:00428028.7APP, MAPT, PRNP, SOD2, TFRC
6protein homodimerization activityGO:00428038.7ADAM10, CAT, CR2, MAPT, TFRC

Sources for Prion Disease

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet