MCID: VRB002
MIFTS: 13

Variably Protease-Sensitive Prionopathy

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Variably Protease-Sensitive Prionopathy

MalaCards integrated aliases for Variably Protease-Sensitive Prionopathy:

Name: Variably Protease-Sensitive Prionopathy 58

Characteristics:

Orphanet epidemiological data:

58
variably protease-sensitive prionopathy
Inheritance: Not applicable;

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

ICD10 via Orphanet 33 A81.8
Orphanet 58 ORPHA454742

Summaries for Variably Protease-Sensitive Prionopathy

MalaCards based summary : Variably Protease-Sensitive Prionopathy is related to prion disease and creutzfeldt-jakob disease. Affiliated tissues include brain.

Wikipedia : 74 Variably protease-sensitive prionopathy (VPSPr) (formerly known as Protease Sensitive Prionopathy) is a... more...

Related Diseases for Variably Protease-Sensitive Prionopathy

Diseases related to Variably Protease-Sensitive Prionopathy via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 12)
# Related Disease Score Top Affiliating Genes
1 prion disease 10.8
2 creutzfeldt-jakob disease 10.8
3 frontotemporal dementia 10.5
4 amyotrophic lateral sclerosis 1 10.4
5 dementia 10.4
6 lateral sclerosis 10.4
7 motor neuron disease 10.4
8 hydrocephalus 10.2
9 normal pressure hydrocephalus 10.2
10 scrapie 10.2
11 myoclonus 10.2
12 inherited prion disease 10.2

Graphical network of the top 20 diseases related to Variably Protease-Sensitive Prionopathy:



Diseases related to Variably Protease-Sensitive Prionopathy

Symptoms & Phenotypes for Variably Protease-Sensitive Prionopathy

Drugs & Therapeutics for Variably Protease-Sensitive Prionopathy

Search Clinical Trials , NIH Clinical Center for Variably Protease-Sensitive Prionopathy

Genetic Tests for Variably Protease-Sensitive Prionopathy

Anatomical Context for Variably Protease-Sensitive Prionopathy

MalaCards organs/tissues related to Variably Protease-Sensitive Prionopathy:

40
Brain

Publications for Variably Protease-Sensitive Prionopathy

Articles related to Variably Protease-Sensitive Prionopathy:

(show all 34)
# Title Authors PMID Year
1
Prion Disease. 61
31533183 2019
2
In Vitro Seeding Activity of Glycoform-Deficient Prions from Variably Protease-Sensitive Prionopathy and Familial CJD Associated with PrPV180I Mutation. 61
30612334 2019
3
Correction to: In Vitro Seeding Activity of Glycoform-Deficient Prions from Variably Protease-Sensitive Prionopathy and Familial CJD Associated with PrPV180I Mutation. 61
30707392 2019
4
Variably protease-sensitive prionopathy mimicking frontotemporal dementia. 61
30847986 2019
5
Variably protease-sensitive prionopathy: A differential diagnostic consideration for dementia. 61
31041129 2019
6
Recent advances in the histo-molecular pathology of human prion disease. 61
30588685 2019
7
Understanding Prion Strains: Evidence from Studies of the Disease Forms Affecting Humans. 61
30934971 2019
8
Variable Protease-Sensitive Prionopathy Transmission to Bank Voles. 61
30561322 2019
9
Variably protease-sensitive prionopathy presenting within ALS/FTD spectrum. 61
30349865 2018
10
[Human prion diseases: current issues]. 61
30040808 2018
11
Variably protease-sensitive prionopathy. 61
29887135 2018
12
Molecular Subtyping of PrPres in Human Sporadic CJD Brain Tissue. 61
28861800 2017
13
Prion diseases. 61
28987186 2017
14
Biochemical Characterization of Prions. 61
28838671 2017
15
A case of variably protease-sensitive prionopathy treated with doxycyclin. 61
25575846 2015
16
Clinical update of Jakob-Creutzfeldt disease. 61
25923128 2015
17
Variably Protease-sensitive Prionopathy in an Apparent Cognitively Normal 93-Year-Old. 61
24845762 2015
18
Variably protease-sensitive prionopathy, a unique prion variant with inefficient transmission properties. 61
25418327 2014
19
Transmission characteristics of variably protease-sensitive prionopathy. 61
25418590 2014
20
The prion protein protease sensitivity, stability and seeding activity in variably protease sensitive prionopathy brain tissue suggests molecular overlaps with sporadic Creutzfeldt-Jakob disease. 61
25331173 2014
21
Concurrent variably protease-sensitive prionopathy and amyotrophic lateral sclerosis. 61
24928712 2014
22
Prions in variably protease-sensitive prionopathy: an update. 61
25437202 2013
23
Variably protease-sensitive prionopathy in the UK: a retrospective review 1991-2008. 61
23550113 2013
24
Small ruminant nor98 prions share biochemical features with human gerstmann-sträussler-scheinker disease and variably protease-sensitive prionopathy. 61
23826096 2013
25
Glycoform-selective prion formation in sporadic and familial forms of prion disease. 61
23527023 2013
26
Coexistence of protease sensitive and resistant prion protein in 129VV homozygous sporadic Creutzfeldt-Jakob disease: a case report. 61
23057723 2012
27
Isolation of soluble and insoluble PrP oligomers in the normal human brain. 61
23070047 2012
28
Sporadic human prion diseases: molecular insights and diagnosis. 61
22710755 2012
29
Human prion diseases in the Netherlands (1998-2009): clinical, genetic and molecular aspects. 61
22558438 2012
30
An overview of human prion diseases. 61
22196171 2011
31
Variably protease-sensitive prionopathy: a novel disease of the prion protein. 61
21584652 2011
32
Insoluble cellular prion protein and its association with prion and Alzheimer diseases. 61
21847014 2011
33
Variably protease-sensitive prionopathy in a PRNP codon 129 heterozygous UK patient with co-existing tau, α synuclein and Aβ pathology. 61
21046409 2010
34
Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. 61
20695009 2010

Variations for Variably Protease-Sensitive Prionopathy

Expression for Variably Protease-Sensitive Prionopathy

Search GEO for disease gene expression data for Variably Protease-Sensitive Prionopathy.

Pathways for Variably Protease-Sensitive Prionopathy

GO Terms for Variably Protease-Sensitive Prionopathy

Sources for Variably Protease-Sensitive Prionopathy

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
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43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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