MCID: NDL013
MIFTS: 56

Nodular Regenerative Hyperplasia malady

Rare diseases, Liver diseases categories
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Summaries for Nodular Regenerative Hyperplasia

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65Wikipedia, 33MalaCards
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Wikipedia:65 Nodular regenerative hyperplasia is a form of liver hyperplasia associated with portal hypertension. more...

MalaCards: Nodular Regenerative Hyperplasia, also known as nodular regenerative hyperplasia of the liver, is related to inflammatory bowel disease and systemic lupus erythematosus, and has symptoms including chronic arterial hypertensionand hepatocellular liver disease/hepatic failure. An important gene associated with Nodular Regenerative Hyperplasia is GFER (growth factor, augmenter of liver regeneration), and among its related pathways are Notch signaling pathway and Pre-NOTCH Expression and Processing. The compounds purine analogues and 6 thioguanine have been mentioned in the context of this disorder. Affiliated tissues include liver, bone marrow and bone, and related mouse phenotypes are liver/biliary system and tumorigenesis.

Aliases & Classifications for Nodular Regenerative Hyperplasia

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43NIH Rare Diseases, 49Orphanet, 26ICD10 via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Liver diseases
Orphanet: 49 
Rare hepatic diseases


Characteristics (Orphanet epidemiological data):

49

Aliases & Descriptions:

nodular regenerative hyperplasia 43
nodular regenerative hyperplasia of the liver 43 49
non-cirrhotic nodulation 43 49
non-cirrhotic nodular transformation 43
miliary hepatocellular adenomatosis 43
non-cirrhotic portal hypertension 43


External Ids:

ICD10 via Orphanet26 K76.8

Related Diseases for Nodular Regenerative Hyperplasia

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17GeneCards, 18GeneDecks
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Diseases related to Nodular Regenerative Hyperplasia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 128)
idRelated DiseaseScoreTop Affiliating Genes
1inflammatory bowel disease31.0TPMT, ITPA, IL6
2systemic lupus erythematosus30.7F3, TPMT, APOH, IL6
3hepatocellular carcinoma30.7GGT1, PCNA, IL6R, IL6, JAG1, NOTCH1
4castleman's disease30.7IL6, IL6R
5cholestasis30.6GGT1, NOTCH2, JAG1
6pancreatitis30.6TPMT, ITPA, IL6, GGT1
7protein s deficiency30.4APOH, F3
8rheumatic disease30.4IL6R, TPMT, APOH
9antiphospholipid syndrome30.4F3, APOH
10autoimmune hepatitis30.4TPMT, GGT1
11connective tissue disease30.3IL6, F3, APOH
12ulcerative colitis30.3IL6R, TPMT, ITPA, IL6
13multiple sclerosis30.3IL6R, IL6, JAG1, TPMT
14rheumatoid arthritis30.3IL6R, TPMT, APOH, IL6
15myeloma30.3NOTCH1, IL6, IL6R
16breast cancer30.2JAG1, IL6R, PCNA, NOTCH2, NOTCH1
17hypertension11.0
18portal hypertension10.9
19hepatitis10.8
20primary biliary cirrhosis10.6
21peliosis hepatis10.6
22lupus erythematosus10.6
23celiac disease10.5
24felty's syndrome10.5
25liver disease10.5
26hepatopulmonary syndrome10.5
27polyarteritis nodosa10.5
28common variable immunodeficiency10.5
29glomerulonephritis10.5
30membranous glomerulonephritis10.4
31macroglobulinemia10.4
32adenoma10.4
33arthritis10.4
34myasthenia gravis10.4
35vasculitis10.4
36rheumatoid vasculitis10.4
37hepatoportal sclerosis10.4
38dyskeratosis congenita10.3
39membranoproliferative glomerulonephritis10.3
40hypersplenism10.3
41graft versus host disease10.2
42crohn's disease10.2
43sickle cell anemia10.2
44hepatocellular adenoma10.2
45cystinosis10.2
46portal vein thrombosis10.2
47idiopathic pulmonary fibrosis10.2
48sjogren's syndrome10.2
49visceral leishmaniasis10.2
50poems syndrome10.2

Graphical network of the top 20 diseases related to Nodular Regenerative Hyperplasia:



Diseases related to nodular regenerative hyperplasia

Symptoms for Nodular Regenerative Hyperplasia

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49Orphanet
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Symptoms:

49
  • chronic arterial hypertension
  • hepatocellular liver disease/hepatic failure

Drugs & Therapeutics for Nodular Regenerative Hyperplasia

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42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

Search ClinicalTrials for Nodular Regenerative Hyperplasia

Search NIH Clinical Center for Nodular Regenerative Hyperplasia

Genetic Tests for Nodular Regenerative Hyperplasia

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Anatomical Context for Nodular Regenerative Hyperplasia

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33MalaCards
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MalaCards organs/tissues related to Nodular Regenerative Hyperplasia:

33
Liver, Bone marrow, Bone, Fetal liver, Endothelial, B cells, T cells, Myeloid, Testes, Skin, Breast

Animal Models for Nodular Regenerative Hyperplasia or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Nodular Regenerative Hyperplasia:

37 (show all 15)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053708.3JAG1, IL6, IL6R, NOTCH2, NOTCH1
2MP:00020068.3F3, IL6, IL6R, NOTCH2, NOTCH1
3MP:00028738.1JAG1, F3, IL6R, NOTCH2, NOTCH1
4MP:00053898.1GGT1, NOTCH2, PCNA, ITPA, TPMT
5MP:00053698.1JAG1, F3, ITPA, IL6, NOTCH2, NOTCH1
6MP:00053857.8JAG1, F3, ITPA, IL6, NOTCH2, NOTCH1
7MP:00053847.6JAG1, F3, ITPA, IL6, IL6R, NOTCH2
8MP:00053917.5JAG1, F3, IL6, NOTCH2, NOTCH1, GGT1
9MP:00107717.3JAG1, F3, ITPA, IL6, NOTCH2, NOTCH1
10MP:00053797.2JAG1, IL6, IL6R, PCNA, NOTCH2, NOTCH1
11MP:00053876.9F3, IL6, IL6R, PCNA, NOTCH2, NOTCH1
12MP:00053766.6APOH, TPMT, JAG1, F3, IL6, IL6R
13MP:00053976.2GGT1, TPMT, JAG1, F3, ITPA, IL6
14MP:00053786.1ITPA, F3, JAG1, TPMT, IL6, IL6R
15MP:00107685.8APOH, TPMT, JAG1, F3, ITPA, IL6

Publications for Nodular Regenerative Hyperplasia

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52PubMed
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Articles related to Nodular Regenerative Hyperplasia:

(show top 50)    (show all 213)
idTitleAuthorsYear
1
Familial occurrence of nodular regenerative hyperplasia of the liver. (23287953)
2013
2
Lengthy follow-up after liver transplantation for nodular regenerative hyperplasia in human immunodeficiency virus-infected patients: does the disease recur? (24296606)
2013
3
Hepatic hemodynamics in 24 patients with nodular regenerative hyperplasia and symptomatic portal hypertension. (22554152)
2012
4
The role of nucleoside and nucleotide analogues in nodular regenerative hyperplasia in HIV-infected patients: a case control study. (21056493)
2011
5
Hepatic encephalopathy as an unusual late complication of transjugular intrahepatic portosystemic shunt insertion for non-cirrhotic portal hypertension caused by nodular regenerative hyperplasia in an HIV-positive patient on highly active antiretroviral therapy. (20029067)
2010
6
Absence of nodular regenerative hyperplasia after low-dose 6-thioguanine maintenance therapy in inflammatory bowel disease patients. (18083079)
2008
7
Nodular regenerative hyperplasia of liver. (18417867)
2008
8
Histopathology of liver biopsies from a thiopurine-naA^ve inflammatory bowel disease cohort: prevalence of nodular regenerative hyperplasia. (18415755)
2008
9
Nodular regenerative hyperplasia in patients with inflammatory bowel disease treated with azathioprine. (17504943)
2007
10
Thioguanin-induced nodular regenerative hyperplasia of the liver-ROC analysis of different MR techniques. (17221208)
2007
11
Nodular regenerative hyperplasia and focal nodular hyperplasia of the liver mimicking hepatic metastasis in children with solid tumors and a review of literature. (17613871)
2007
12
Nodular regenerative hyperplasia: not all nodules are created equal. (16799965)
2006
13
Celiac disease associated with nodular regenerative hyperplasia, pulmonary abnormalities, and IgA anticardiolipin antibodies. (16394874)
2006
14
Concomitant hepatocellular carcinoma and non-Hodgkin's lymphoma in a patient with nodular regenerative hyperplasia. (16669877)
2006
15
Liver transplantation in precirrhotic biliary tract disease: Portal hypertension is frequently associated with nodular regenerative hyperplasia and obliterative portal venopathy. (17063088)
2006
16
Nodular regenerative hyperplasia: a reversible entity associated with azathioprine therapy. (16607155)
2006
17
Nodular regenerative hyperplasia of the liver and Hodgkin's disease: a case report. (15727082)
2004
18
Nodular regenerative hyperplasia of the liver: an under-recognized cause of portal hypertension in hematological disorders. (15054815)
2004
19
Nodular regenerative hyperplasia of the liver and coeliac disease: potential role of IgA anticardiolipin antibody. (15194657)
2004
20
Nodular regenerative hyperplasia of the liver: a case report. (14593541)
2003
21
Nodular regenerative hyperplasia of the liver. A review of 14 cases. (11677979)
2001
22
The liver in collagen diseases: pathologic study of 160 cases with particular reference to hepatic arteritis, primary biliary cirrhosis, autoimmune hepatitis and nodular regenerative hyperplasia of the liver. (11092254)
2000
23
Nodular regenerative hyperplasia of the liver: case report of a 13-year-old girl and review of the literature. (10663513)
2000
24
Results of liver transplantation for nodular regenerative hyperplasia. (11090022)
2000
25
Nodular regenerative hyperplasia of the liver and antiphospholipid antibodies: report of two cases and review of the literature. (10192512)
1999
26
Nodular regenerative hyperplasia of the liver: description of two cases. (8544802)
1996
27
Childhood nodular regenerative hyperplasia of the liver complicated by severe hypoxemia. (7608831)
1995
28
Nodular regenerative hyperplasia of the liver following orthotopic liver transplantation. (7652907)
1995
29
A peculiar distribution of peroxisomes in a patient with nodular regenerative hyperplasia of the liver. (8014452)
1994
30
Nodular regenerative hyperplasia of the liver and primary SjAPgren's syndrome. (8151575)
1994
31
Nodular regenerative hyperplasia of the liver: ultrasonographic appearance and echo-guided bioptic diagnosis. (7812029)
1994
32
Nodular regenerative hyperplasia: a controversial indication for orthotopic liver transplantation. (7916934)
1994
33
Hepatic infarction and nodular regenerative hyperplasia of the liver with associated anticardiolipin antibodies in a young woman. (8071516)
1994
34
Diffuse nodular regenerative hyperplasia of the liver associated with human immunodeficiency virus and visceral leishmaniasis. (8438854)
1993
35
Azathioprine induced liver disease: nodular regenerative hyperplasia of the liver and perivenous fibrosis in a patient treated for multiple sclerosis. (2060883)
1991
36
Nodular regenerative hyperplasia: a cause of ascites and hepatomegaly after chemotherapy for leukemia. (1986559)
1991
37
Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2,500 autopsies and a new classification of benign hepatocellular nodules. (2189821)
1990
38
Nodular regenerative hyperplasia mimicking cirrhosis of the liver. (2379880)
1990
39
Nodular regenerative hyperplasia of the liver, peliosis hepatis, and perisinusoidal fibrosis. Association with angioimmunoblastic lymphadenopathy and severe hypoxemia. (2344932)
1990
40
Peliosis hepatis, nodular regenerative hyperplasia of the liver, and light-chain deposition in a patient with WaldenstrAPm's macroglobulinemia. (3134270)
1988
41
Nodular regenerative hyperplasia of the liver with and without portal hypertension: a comparison. (3963734)
1986
42
Systemic lupus erythematosus and nodular regenerative hyperplasia of the liver. (3947146)
1986
43
Nodular regenerative hyperplasia and focal nodular hyperplasia of the liver associated with severe cholestasis. Report of a case with autopsy findings. (3713453)
1986
44
Nodular regenerative hyperplasia of the liver in a patient with multiple myeloma and systemic amyloidosis. (2416186)
1985
45
Nodular regenerative hyperplasia of the liver. Report of a case and review of the literature. (4047265)
1985
46
Nodular regenerative hyperplasia of the liver associated with progressive systemic sclerosis: a case report with ultrastructural observation. (6644697)
1983
47
Nodular regenerative hyperplasia of the liver with membranous glomerulonephritis. (7301692)
1981
48
Nodular regenerative hyperplasia of the liver following renal transplantation. (7016482)
1981
49
Nodular regenerative hyperplasia of the liver. (6945840)
1981
50
Nodular regenerative hyperplasia of the liver in hematologic disorders: a possible response to obliterative portal venopathy. A morphometric study of nine cases with an hypothesis on the pathogenesis. (7432153)
1980

Variations for Nodular Regenerative Hyperplasia

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Expression for genes affiliated with Nodular Regenerative Hyperplasia

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Nodular Regenerative Hyperplasia

Search GEO for disease gene expression data for Nodular Regenerative Hyperplasia.

Pathways for genes affiliated with Nodular Regenerative Hyperplasia

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50PathCards, 30KEGG, 57SinoBiological, 55Reactome, 38NCBI BioSystems Database, 60Thomson Reuters, 5Cell Signaling Technology, 54R&D Systems, 53QIAGEN
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Pathways related to Nodular Regenerative Hyperplasia according to GeneCards/GeneDecks:

(show all 16)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.7NOTCH1, JAG1
2
Show member pathways
9.5NOTCH1, NOTCH2
39.5NOTCH2, NOTCH1
49.5IL6R, IL6
59.5IL6R, IL6
69.3IL6, NOTCH1
79.2NOTCH1, NOTCH2, JAG1
8
Show member pathways
9.2JAG1, NOTCH2, NOTCH1
99.2NOTCH1, NOTCH2, JAG1
10
Show member pathways
9.2NOTCH1, NOTCH2, JAG1
11
Show member pathways
Delta-Notch Signaling Pathway38
Notch Signaling Pathway38
9.2NOTCH1, NOTCH2, JAG1
12
Show member pathways
Notch signaling pathway38
9.2NOTCH1, NOTCH2, JAG1
139.2JAG1, NOTCH2, NOTCH1
149.0IL6, IL6R, PCNA
15
Show member pathways
8.3GFER, IL6, IL6R, NOTCH2, NOTCH1
16
Show member pathways
8.3GFER, IL6, IL6R, NOTCH2, NOTCH1

Compounds for genes affiliated with Nodular Regenerative Hyperplasia

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51PharmGKB, 45Novoseek, 24HMDB, 11DrugBank, 29IUPHAR, 3BitterDB, 61Tocris Bioscience
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Compounds related to Nodular Regenerative Hyperplasia according to GeneCards/GeneDecks:

(show top 50)    (show all 62)
idCompoundScoreTop Affiliating Genes
1purine analogues5110.3ITPA, TPMT
26 thioguanine4510.2TPMT, ITPA
3thiopurine4510.2ITPA, TPMT
46 mercaptopurine4510.2ITPA, TPMT
5inosine triphosphate45 2411.1ITPA, TPMT
6kaolin4510.0APOH, F3
7coumarins4510.0APOH, F3
8tocilizumab45 1110.9IL6, IL6R
9ticlopidine45 1110.9APOH, F3
10ptca459.9APOH, F3
11inosine45 2410.8TPMT, ITPA
12warfarin45 51 24 1112.7TPMT, F3, APOH
13rituximab45 51 1111.5APOH, IL6R, IL6
14diethylnitrosamine459.5PCNA, GGT1
15dextran sulfate459.5IL6, F3, APOH
16leflunomide45 51 1111.4PCNA, IL6
17aspirin45 51 29 2412.4PCNA, APOH, F3
18endotoxin459.4F3, APOH, IL6
19hcec459.4IL6, GGT1
20diethyl dithiocarbamate459.3TPMT, GGT1
21lycopene45 2410.3PCNA, GGT1
22prednisolone45 29 1111.3IL6R, IL6, TPMT, APOH
23cyclophosphamide45 51 1111.2F3, PCNA, TPMT, APOH
24propylthiouracil45 3 1111.2GGT1, PCNA, APOH
25vitamin b12459.2GGT1, APOH, PCNA
26betacarotene459.2PCNA, GGT1, APOH
27fibrinogen459.1F3, IL6R, IL6, APOH
28histamine45 29 2411.1TPMT, IL6R, IL6, PCNA
29epinephrine45 24 1111.1IL6, PCNA, F3
30cyclosporin a45 29 6111.1IL6R, PCNA, IL6, TPMT
31arsenite45 2410.0PCNA, F3, GGT1
32pentoxifylline45 1110.0F3, IL6, GGT1
33tacrolimus45 51 1110.8F3, TPMT, GGT1, PCNA
34heparin45 29 24 1111.7GGT1, APOH, F3, PCNA
35azathioprine45 3 51 1111.6PCNA, GGT1, TPMT, APOH, ITPA
36cholesterol45 29 24 1111.6IL6R, APOH, IL6, GGT1
37n acetylcysteine458.6GGT1, IL6, PCNA
38arginine458.6APOH, IL6R, GGT1, PCNA
39aspartate458.5F3, PCNA, NOTCH1, GGT1
40lactate458.5IL6, PCNA, GGT1, APOH
41indomethacin45 29 61 1111.5IL6, PCNA, GGT1
42testosterone45 61 24 1111.5IL6, GGT1, IL6R, PCNA
43retinoic acid45 249.4PCNA, NOTCH1, F3, IL6, IL6R
44nitric oxide45 24 1110.4JAG1, NOTCH1, IL6, APOH
45lipid458.1GGT1, NOTCH1, IL6R, IL6, APOH
46alanine458.1GGT1, PCNA, F3, IL6, TPMT
47vegf458.1APOH, IL6, IL6R, PCNA, NOTCH1, F3
48dexamethasone45 51 29 1111.1NOTCH1, IL6R, GGT1, PCNA, IL6
49methotrexate51 45 119.6IL6R, GGT1, PCNA, APOH, TPMT, ITPA
50creatinine457.5PCNA, GGT1, APOH, TPMT, F3, IL6R

GO Terms for genes affiliated with Nodular Regenerative Hyperplasia

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16Gene Ontology
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Cellular components related to Nodular Regenerative Hyperplasia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1interleukin-6 receptor complexGO:0058969.2IL6, IL6R
2cell surfaceGO:0099868.3NOTCH1, NOTCH2, IL6R, F3, APOH
3extracellular regionGO:0055768.0NOTCH1, NOTCH2, IL6R, IL6, JAG1, GFER

Biological processes related to Nodular Regenerative Hyperplasia according to GeneCards/GeneDecks:

(show all 33)
idNameGO IDScoreTop Affiliating Genes
1morphogenesis of an epithelial sheetGO:00201110.1JAG1, NOTCH2
2distal tubule developmentGO:07201710.0NOTCH1, JAG1
3negative regulation of stem cell differentiationGO:200073710.0JAG1, NOTCH1
4cell fate determinationGO:00170910.0JAG1, NOTCH2
5cardiac septum morphogenesisGO:06041110.0NOTCH1, JAG1
6response to muramyl dipeptideGO:03249510.0NOTCH1, JAG1
7neuronal stem cell maintenanceGO:09715010.0NOTCH1, JAG1
8interleukin-4 secretionGO:0726029.9NOTCH2, NOTCH1
9hepatic immune responseGO:0023849.9IL6, IL6R
10negative regulation of collagen biosynthetic processGO:0329669.9IL6R, IL6
11neutrophil mediated immunityGO:0024469.9IL6R, IL6
12interleukin-6-mediated signaling pathwayGO:0701029.9IL6R, IL6
13positive regulation of leukocyte chemotaxisGO:0026909.8IL6R, IL6
14inflammatory response to antigenic stimulusGO:0024379.8NOTCH2, NOTCH1
15positive regulation of chemokine productionGO:0327229.8IL6, IL6R
16monocyte chemotaxisGO:0025489.7IL6, IL6R
17bone remodelingGO:0468499.7IL6, NOTCH2
18defense response to Gram-negative bacteriumGO:0508299.7IL6R, IL6
19positive regulation of tyrosine phosphorylation of Stat3 proteinGO:0425179.7IL6R, IL6
20negative regulation of fat cell differentiationGO:0455999.7IL6, JAG1
21defense response to Gram-positive bacteriumGO:0508309.7IL6, IL6R
22pulmonary valve morphogenesisGO:0031849.6NOTCH1, NOTCH2, JAG1
23Notch signaling involved in heart developmentGO:0613149.6JAG1, NOTCH2, NOTCH1
24Notch receptor processingGO:0072209.6NOTCH1, NOTCH2, JAG1
25positive regulation of JAK-STAT cascadeGO:0464279.6NOTCH1, IL6
26acute-phase responseGO:0069539.6IL6R, IL6
27positive regulation of interleukin-6 productionGO:0327559.6IL6, IL6R
28Notch signaling pathwayGO:0072199.6JAG1, NOTCH2, NOTCH1
29endocrine pancreas developmentGO:0310189.5IL6, IL6R
30positive regulation of smooth muscle cell proliferationGO:0486619.5IL6R, IL6
31humoral immune responseGO:0069599.3IL6, NOTCH2, NOTCH1
32determination of left/right symmetryGO:0073689.2NOTCH1, NOTCH2
33positive regulation of apoptotic processGO:0430658.8NOTCH1, NOTCH2, IL6

Molecular functions related to Nodular Regenerative Hyperplasia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1growth factor activityGO:0080839.3IL6, JAG1, GFER
2interleukin-6 receptor bindingGO:0051389.2IL6R, IL6
3protein bindingGO:0055155.7IL6, F3, APOH, IL6R, PCNA, NOTCH2

Products for genes affiliated with Nodular Regenerative Hyperplasia

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Sources for Nodular Regenerative Hyperplasia

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet