PPMS
MCID: PRM108
MIFTS: 45

Primary Progressive Multiple Sclerosis (PPMS) malady

Summaries for Primary Progressive Multiple Sclerosis

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8Disease Ontology, 32MalaCards
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Disease Ontology:8 A multiple sclerosis that is characterized by steady worsening of neurologic functioning, without any distinct relapses or periods of remission. the rate of progression may vary over time, with occasional plateaus or temporary improvements, but the progression is continuous.

MalaCards: Primary Progressive Multiple Sclerosis, also known as multiple sclerosis, primary progressive, is related to multiple sclerosis and neuromyelitis optica. An important gene associated with Primary Progressive Multiple Sclerosis is MOG (myelin oligodendrocyte glycoprotein), and among its related pathways are Rheumatoid arthritis and Cytokines and Inflammatory Response. The compounds ribavirin and cycloheximide have been mentioned in the context of this disorder. Affiliated tissues include t cells, and related mouse phenotypes are vision/eye and skeleton.

Aliases & Classifications for Primary Progressive Multiple Sclerosis

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8Disease Ontology, 44Novoseek, 60UMLS
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Aliases & Descriptions:

primary progressive multiple sclerosis 8 44
multiple sclerosis, primary progressive 60
primary-progressive ms 8
ppms 8


External Ids:

Disease Ontology8 DOID:0050784

Related Diseases for Primary Progressive Multiple Sclerosis

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Sources:
17GeneCards, 18GeneDecks
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Diseases in the Multiple Sclerosis family:

primary progressive multiple sclerosis Multiple Sclerosis 4
Multiple Sclerosis 3 Multiple Sclerosis 1
Multiple Sclerosis 2 Multiple Sclerosis 5

Diseases related to Primary Progressive Multiple Sclerosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 187)
idRelated DiseaseScoreTop Affiliating Genes
1multiple sclerosis31.2HLA-DRB1, HLA-DQB1
2neuromyelitis optica30.2MOG, MBP, IFNB1
3adrenoleukodystrophy30.2MOG, PLP1, MBP
4relapsing-remitting multiple sclerosis30.2BDNF, MOG, MX1, MMP9, IL10, MBP
5asthma29.9IFNB1, HLA-DRB1, MBP, MMP9, ICAM1, CASP8
6ppm-x syndrome10.4
7root caries10.3
8progressive relapsing multiple sclerosis10.3
9dental fluorosis10.2
10neurofibromatosis10.1
11cerebritis10.1
12dentin sensitivity10.1
13jamaican vomiting sickness10.1
14myocarditis10.0IFNB1
15neuritis10.0MBP
16neuropathy10.0MBP
17polyneuropathy10.0MBP
18lupus erythematosus10.0MX1
19chronic inflammatory demyelinating polyradiculoneuropathy10.0IFNB1
20periodontitis10.0MMP9
21lung cancer10.0MMP9
22meningitis10.0MMP9
23lyme disease10.0MBP
24rabies10.0MX1
25biliary atresia10.0MX1
26kawasaki disease10.0MMP9
27prion disease10.0MBP
28interstitial lung disease10.0MMP9
29cocaine abuse10.0PLP1
30diabetic ketoacidosis10.0CTLA4
31genetic prion diseases10.0PRNP
32gastritis10.0PRNP
33myopathy10.0PRNP
34acute disseminated encephalomyelitis10.0MOG, MBP
35leishmaniasis10.0IL10
36pulmonary fibrosis10.0MMP9
37aphasia10.0GRN
38hypothyroidism10.0CTLA4
39myeloid leukemia10.0CASP8
40wegener's granulomatosis10.0CTLA4
41acute myocardial infarction10.0MMP9
42common variable immune deficiency10.0IL10
43schistosomiasis10.0IL10
44leprosy10.0HLA-DRB1
45neutropenia10.0IFNB1
46carotid stenosis10.0MMP9
47acute leukemia10.0CASP8
48secondary progressive multiple sclerosis10.0MBP, IFNB1
49pulmonary disease, chronic obstructive10.0MMP9
50pemphigus foliaceus10.0HLA-DRB1

Graphical network of the top 20 diseases related to Primary Progressive Multiple Sclerosis:



Diseases related to primary progressive multiple sclerosis

Clinical Features for Primary Progressive Multiple Sclerosis

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Drugs & Therapeutics for Primary Progressive Multiple Sclerosis

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Primary Progressive Multiple Sclerosis

Drug clinical trials:

Search ClinicalTrials for Primary Progressive Multiple Sclerosis

Search NIH Clinical Center for Primary Progressive Multiple Sclerosis

Search CenterWatch for Primary Progressive Multiple Sclerosis

Genetic Tests for Primary Progressive Multiple Sclerosis

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Anatomical Context for Primary Progressive Multiple Sclerosis

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32MalaCards
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MalaCards organs/tissues related to Primary Progressive Multiple Sclerosis:

32
T cells

Animal Models for Primary Progressive Multiple Sclerosis or affiliated genes

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Sources:
36MGI
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Publications for Primary Progressive Multiple Sclerosis

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Sources:
50PubMed
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Articles related to Primary Progressive Multiple Sclerosis:

(show all 36)
idTitleAuthorsYear
1
Characteristic cerebrospinal fluid cytokine/chemokine profiles in neuromyelitis optica, relapsing remitting or primary progressive multiple sclerosis. (23637915)
2013
2
Comparison of IFN-I^ inducible gene expression in primary-progressive and relapsing-remitting multiple sclerosis. (24200257)
2013
3
Decreased circulating miRNA levels in patients with primary progressive multiple sclerosis. (24277735)
2013
4
Pediatric multiple sclerosis with primary progressive course--report of a retrospective cohort study in Iran. (23254570)
2013
5
The C609T inborn polymorphism in NAD(P)H:quinone oxidoreductase 1 is associated with susceptibility to multiple sclerosis and affects the risk of development of the primary progressive form of the disease. (21605663)
2011
6
No association between genetic polymorphism at codon 129 of the prion protein gene and primary progressive multiple sclerosis. (21320996)
2011
7
Heterozygous X-linked adrenoleukodystrophy-associated myelopathy mimicking primary progressive multiple sclerosis. (20803344)
2011
8
Lack of replication of KIF1B gene in an Italian primary progressive multiple sclerosis cohort. (20067515)
2010
9
Progranulin gene variability increases the risk for primary progressive multiple sclerosis in males. (20463744)
2010
10
Genetic association of CASP8 polymorphisms with primary progressive multiple sclerosis. (20363033)
2010
11
APOE epsilon status in Hungarian patients with primary progressive multiple sclerosis. (21110240)
2010
12
Does the DRB1*1501 allele confer more severe and faster progression in primary progressive multiple sclerosis patients? HLA in primary progressive multiple sclerosis. (19616314)
2009
13
Tumour necrosis factor alpha gene (TNF-alpha) -376 polymorphism in Hungarian patients with primary progressive multiple sclerosis. (19201038)
2009
14
Voxel-based assessment of differences in damage and distribution of white matter lesions between patients with primary progressive and relapsing-remitting multiple sclerosis. (18268194)
2008
15
MxA protein--an interferon beta biomarker in primary progressive multiple sclerosis patients. (18549400)
2008
16
Cell surface adhesion molecules and cytokine profiles in primary progressive multiple sclerosis. (17613596)
2007
17
Intravenous immunoglobulin in primary and secondary chronic progressive multiple sclerosis: a randomized placebo controlled multicentre study. (17623736)
2007
18
Interferon-beta1b treatment modulates cytokines in patients with primary progressive multiple sclerosis. (17083335)
2006
19
Primary progressive multiple sclerosis as a phenotype of a PLP1 gene mutation. (16130097)
2005
20
Gene expression and genotyping studies implicate the interleukin 7 receptor in the pathogenesis of primary progressive multiple sclerosis. (16075257)
2005
21
Autoantibody synthesis in primary progressive multiple sclerosis patients treated with interferon beta-1b. (15645350)
2004
22
Increased frequencies of serum antibodies to neurofilament light in patients with primary chronic progressive multiple sclerosis. (15584481)
2004
23
Decreased MMP-9 production in primary progressive multiple sclerosis patients. (15327032)
2004
24
Immunologic factors in primary progressive multiple sclerosis. (15218805)
2004
25
Serum gelatinase B/MMP-9 in primary progressive multiple sclerosis patients treated with interferon-beta-1a. (14504963)
2003
26
Interferon-beta-1 b decreased matrix metalloproteinase-9 serum levels in primary progressive multiple sclerosis. (14586607)
2003
27
CTLA4 exon 1 dimorphism is associated with primary progressive multiple sclerosis. (12458055)
2002
28
Frequency of functional interleukin-10 promoter polymorphism is different between relapse-onset and primary progressive multiple sclerosis. (12039409)
2002
29
Monitoring disease activity and progression in primary progressive multiple sclerosis using MRI: sub-voxel registration to identify lesion changes and to detect cerebral atrophy. (11985382)
2002
30
Intracellular cytokine profile in T-cell subsets of multiple sclerosis patients: different features in primary progressive disease. (11475436)
2001
31
Detailed analysis of the oligodendrocyte myelin glycoprotein gene in four patients with neurofibromatosis 1 and primary progressive multiple sclerosis. (10766898)
2000
32
Serum soluble adhesion molecules in multiple sclerosis: raised sVCAM-1, sICAM-1 and sE-selectin in primary progressive disease. (10195402)
1999
33
Immunological profile of patients with primary progressive multiple sclerosis. Expression of adhesion molecules. (10581223)
1999
34
A case of primary SjAPgren's syndrome with CNS disease mimicking chronic progressive multiple sclerosis]. (9868312)
1998
35
Interferon-gamma activated calcium influx in peripheral blood lymphocytes from patients with primary and secondary progressive multiple sclerosis. (8937348)
1996
36
Soluble E-selectin in multiple sclerosis: raised concentrations in patients with primary progressive disease. (8558145)
1996

Genetic Variations for Primary Progressive Multiple Sclerosis

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Expression for genes affiliated with Primary Progressive Multiple Sclerosis

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Primary Progressive Multiple Sclerosis

Search GEO for disease gene expression data for Primary Progressive Multiple Sclerosis.

Pathways for genes affiliated with Primary Progressive Multiple Sclerosis

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29KEGG, 37NCBI BioSystems Database, 51QIAGEN, 53Reactome
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Pathways related to Primary Progressive Multiple Sclerosis according to GeneCards/GeneDecks:

(show all 15)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.2CTLA4
210.1HLA-DRB1
39.7MBP, PLP1
4
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9.6CASP8, HLA-DQB1, HLA-DRB1
59.2IL10, HLA-DRB1, HLA-DQB1, ICAM1
69.2IL10, HLA-DRB1, HLA-DQB1, ICAM1
7
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9.2HLA-DQB1, HLA-DRB1, IL10, BDNF
8
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9.1CASP8, IFNB1, IL10, MMP9
98.8CASP8, IFNB1, HLA-DQB1, HLA-DRB1, IL10
108.7ICAM3, ICAM1, HLA-DQB1, HLA-DRB1
11
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8.6IL10, CASP8, ICAM1, CTLA4, HLA-DQB1, HLA-DRB1
128.6BDNF, ICAM1, MBP, OMG, MMP9
13
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8.6IFNB1, ICAM1, HLA-DQB1, HLA-DRB1, IL7R, MX1
14
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8.5CASP8, IFNB1, ICAM1, HLA-DQB1, HLA-DRB1, MX1
15
Hide members
7.2CASP8, IFNB1, ICAM3, ICAM1, CTLA4, HLA-DQB1

Compounds for genes affiliated with Primary Progressive Multiple Sclerosis

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Sources:
44Novoseek, 49PharmGKB, 11DrugBank, 59Tocris Bioscience, 28IUPHAR, 24HMDB
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Compounds related to Primary Progressive Multiple Sclerosis according to GeneCards/GeneDecks:

(show top 50)    (show all 93)
idCompoundScoreTop Affiliating Genes
1ribavirin44 49 1112.1MX1, IL10, MMP9
2cycloheximide449.9IFNB1, MX1, CASP8
3glutamate449.9PLP1, MBP, PRNP
4helenalin449.9CASP8, ICAM1, MMP9
5betaseron449.8MX1, IFNB1, IL10
6plp (139-151)599.8PRNP, MBP, MOG
7mog (35-55)599.8PRNP, MBP, MOG
8c3bot (154-182)599.8PRNP, MBP, MOG
9nogo-66 (1-40)599.8MBP, MOG, PRNP
10ono 2506599.8PRNP, MBP, MOG
112,3-cyclic nucleotide449.8PLP1, MBP, MOG
12aspartate449.8APOE, BDNF, PRNP
13serine449.7MX1, MMP9, CTLA4, MOG, PRNP
14amino acid449.7BDNF, PRNP, HLA-DRB1
15hemocyanin449.6PRNP, CTLA4, MBP
16natalizumab44 1110.5IL10, ICAM1, IFNB1
17cyclosporin a44 28 5911.5CTLA4, MBP, ICAM1
18imiquimod44 28 59 1112.5IFNB1, IL10, MX1
19ifn-alpha449.5MX1, IL10, CASP8, IFNB1
20hcec449.4BDNF, IFNB1, ICAM1
21ccl344 2810.4MMP9, ICAM1, IFNB1, IL10
22neopterin449.4MX1, IL10, IFNB1, ICAM1
23tetracycline44 1110.3MMP9, PRNP, IFNB1, CASP8
24edss449.3MBP, IFNB1, APOE
25tgf beta1449.2IFNB1, CTLA4, MMP9, IL10, CASP8
26ly294002449.2MMP9, IFNB1, CASP8, CTLA4, ICAM1
27rantes449.2IFNB1, ICAM3, ICAM1
28acetylcholine44 49 28 11 2413.2PLP1, BDNF, MOG, CTLA4, MBP
29ivig449.1IFNB1, MBP, IL10, MMP9
30ibuprofen44 28 49 11 2413.1BDNF, APOE, IL10, IFNB1
31silver449.0BDNF, MOG, IFNB1, MBP, PLP1
32sb 20358044 599.9CASP8, IFNB1, ICAM1, PRNP, IL10, MMP9
33methionine448.8BDNF, PLP1, PRNP, CTLA4, IFNB1
34atorvastatin44 49 28 11 2412.8MMP9, IL10, ICAM1, APOE
35h2o2448.8MBP, ICAM1, IFNB1, CASP8, PRNP
36simvastatin44 49 59 28 11 2413.7IL10, MMP9, APOE, ICAM1, IFNB1, CASP8
37dexamethasone44 49 28 1111.6CASP8, CTLA4, MMP9, IFNB1, ICAM3
38methotrexate44 49 1110.6HLA-DRB1, MBP, IFNB1, IL10, MMP9, CTLA4
39actinomycin d448.5ICAM1, APOE, MMP9, IFNB1, BDNF, CASP8
40n acetylcysteine448.3APOE, MMP9, PLP1, ICAM1, CASP8, BDNF
41thymidine44 249.3IL10, PLP1, CTLA4, MBP, IFNB1, BDNF
42vegf448.2BDNF, APOE, GRN, IL10, ICAM1, MMP9
43polysaccharide448.1IFNB1, PRNP, ICAM1, CASP8, ICAM3
44glatiramer acetate44 119.1PLP1, IL10, HLA-DRB1, CTLA4, MBP, IFNB1
45phosphatidylinositol448.0ICAM3, IL7R, CASP8, MMP9, ICAM1, BDNF
46cysteine448.0APOE, CASP8, GRN, CTLA4, MMP9, PLP1
47retinoic acid44 248.8IFNB1, PLP1, MMP9, MBP, ICAM1, BDNF
48lipid447.7PRNP, CTLA4, APOE, BDNF, MBP, IFNB1
49alanine447.7APOE, MX1, CTLA4, HLA-DRB1, PRNP, CASP8
50tyrosine446.3IL7R, MMP9, HLA-DRB1, CTLA4, MBP, ICAM1

GO Terms for genes affiliated with Primary Progressive Multiple Sclerosis

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Sources:
16Gene Ontology
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Cellular components related to Primary Progressive Multiple Sclerosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1external side of plasma membraneGO:0098979.0ICAM1, CTLA4, HLA-DRB1, IL7R
2extracellular spaceGO:0056158.3MMP9, IL10, GRN, APOE, ICAM1, IFNB1
3plasma membraneGO:0058866.4IL7R, MOG, ICAM3, ICAM1, MBP, APOE

Biological processes related to Primary Progressive Multiple Sclerosis according to GeneCards/GeneDecks:

(show all 12)
idNameGO IDScoreTop Affiliating Genes
1immunoglobulin production involved in immunoglobulin mediated immune responseGO:00238110.1HLA-DQB1, HLA-DRB1
2humoral immune response mediated by circulating immunoglobulinGO:0024559.9HLA-DQB1, HLA-DRB1
3response to copper ionGO:0466889.8PRNP, ICAM1
4axon ensheathmentGO:0083669.8MBP, PLP1
5negative regulation of interferon-gamma productionGO:0326899.8HLA-DRB1, PRNP, IL10
6T cell costimulationGO:0312959.7HLA-DRB1, HLA-DQB1, CTLA4
7B cell proliferationGO:0421009.7IL7R, IL10, IFNB1
8interferon-gamma-mediated signaling pathwayGO:0603339.7HLA-DRB1, HLA-DQB1, ICAM1
9negative regulation of B cell proliferationGO:0308899.6IL10, CTLA4
10response to ethanolGO:0454719.2APOE, ICAM1, CASP8
11cytokine-mediated signaling pathwayGO:0192219.0MX1, HLA-DRB1, HLA-DQB1, ICAM1, IFNB1
12immune responseGO:0069559.0IL7R, HLA-DRB1, HLA-DQB1, CTLA4, MBP

Molecular functions related to Primary Progressive Multiple Sclerosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1MHC class II receptor activityGO:0323959.9HLA-DRB1, HLA-DQB1
2structural constituent of myelin sheathGO:0199119.4PLP1, MBP

Products for genes affiliated with Primary Progressive Multiple Sclerosis

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Sources for Primary Progressive Multiple Sclerosis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet