PAH
MCID: PRM021
MIFTS: 74

Primary Pulmonary Hypertension (PAH) malady

Cardiovascular, Respiratory, Genetic categories

Summaries for Primary Pulmonary Hypertension

Sources:
21Genetics Home Reference, 43NIH Rare Diseases, 34MedlinePlus, 3CDC, 64Wikipedia, 47OMIM, 33MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
NIH Rare Diseases:43 Pulmonary arterial hypertension (pah) is a progressive condition that affects the heart and lungs. it is characterized by abnormally high blood pressure (hypertension) in the pulmonary artery, the blood vessel that carries blood from the heart to the lungs. the most common signs and symptoms are shortness of breath (dyspnea) during exertion and fainting spells. as the condition worsens, people can experience dizziness, swelling (edema) of the ankles or legs, chest pain, and a racing pulse. most cases of pah occur in individuals with no family history of the disorder. although some cases are due to mutations in the bmpr2 gene and inherited in an autosomal dominant pattern, a gene mutation has not yet been identified in most individuals. when pah is inherited from an affected relative it is called "familial" pah. cases with no identifiable cause may be referred to as "idiopathic" pah. pah can also occur secondary to an underlying disorder such as connective tissue diseases, hiv infection, chronic hemolytic anemia, and congenital heart disease, to name a few. pah can also be induced by certain drugs and toxins, for example fenfluramine and dexfenfluramine (appetite suppressants now banned by the fda), toxic rapeseed oil, and amphetamines.  last updated: 9/20/2013

MalaCards: Primary Pulmonary Hypertension, also known as pulmonary hypertension, is related to pulmonary disease, chronic obstructive and mixed connective tissue disease. An important gene associated with Primary Pulmonary Hypertension is BMPR2 (bone morphogenetic protein receptor, type II (serine/threonine kinase)), and among its related pathways are Common Pathway and TOB in Osteoblast Signaling. The drugs alprostadil and epoprostenol and the compounds captopril and norepinephrine have been mentioned in the context of this disorder. Affiliated tissues include whole blood, brain and heart, and related mouse phenotypes are muscle and endocrine/exocrine gland.

Genetics Home Reference:21 Pulmonary arterial hypertension is a progressive disorder characterized by abnormally high blood pressure (hypertension) in the pulmonary artery, the blood vessel that carries blood from the heart to the lungs. Hypertension occurs when most of the very small arteries throughout the lungs narrow in diameter, which increases the resistance to blood flow through the lungs. To overcome the increased resistance, pressure increases in the pulmonary artery and in the heart chamber that pumps blood into the pulmonary artery (the right ventricle).

MedlinePlus:34 Pulmonary hypertension (ph) is high blood pressure in the arteries to your lungs. it is a serious condition. if you have it, the blood vessels that carry blood from your heart to your lungs become hard and narrow. your heart has to work harder to pump the blood through. over time, your heart weakens and cannot do its job and you can develop heart failure. symptoms of ph include shortness of breath during routine activity, such as climbing two flights of stairs tiredness chest pain a racing heartbeat pain on the upper right side of the abdomen decreased appetite as ph worsens, you may find it hard to do any physical activities. there are two main kinds of ph. one runs in families or appears for no known reason. the other kind is related to another condition, usually heart or lung disease. there is no cure for ph. treatments can control symptoms. they involve treating the heart or lung disease, medicines, oxygen, and sometimes lung transplantation. nih: national heart, lung, and blood institute

CDC:3 Symptoms of pulmonary hypertension include—

Wikipedia:64 Pulmonary hypertension (PH) is an increase of blood pressure in the pulmonary artery, pulmonary vein, or... more...

Description from OMIM:47 265400,178600

Aliases & Classifications for Primary Pulmonary Hypertension

Sources:
8Disease Ontology, 64Wikipedia, 22GTR, 21Genetics Home Reference, 10DISEASES, 45Novoseek, 34MedlinePlus, 61UMLS, 43NIH Rare Diseases, 49Orphanet, 27ICD9CM, 47OMIM, 57SNOMED-CT, 35MeSH, 40NCIt, 26ICD10 via Orphanet, 25ICD10
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Genetic
Anatomical: Cardiovascular, Respiratory


Characteristics (Orphanet epidemiological data):

49
idiopathic pulmonary arterial hypertension:
Inheritance: Sporadic; Prevalence: 1-9/1000000; Age of onset: Variable; Age of death: Adult


Aliases & Descriptions:

primary pulmonary hypertension 8 64 22 21 10 45
pulmonary hypertension 8 64 10 34 61
idiopathic pulmonary arterial hypertension 43 49 61
familial primary pulmonary hypertension 64 21 61
sporadic primary pulmonary hypertension 64 21 61
idiopathic pulmonary hypertension 64 21 61
pulmonary arterial hypertension 64 43 21
ayerza syndrome 64 21 61
pph 64 21
hereditary pulmonary arterial hypertension 43
heritable pulmonary arterial hypertension 43
familial pulmonary arterial hypertension 43
pulmonary hypertension, idiopathic 8
hypertension pulmonary 45
fpah 43
fpph 21
ipah 49
ppht 21
pah 21


External Ids:

Disease Ontology8 DOID:14557, DOID:6432
ICD9CM27 416.0
NCIt40 C3120
ICD10 via Orphanet26 I27.0
ICD1025 I27.0

Related Diseases for Primary Pulmonary Hypertension

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the Primary Pulmonary Hypertension family:

pulmonary hypertension, primary, 4 pulmonary hypertension, familial primary, 1, with or without hht
pulmonary hypertension, primary, 3 pulmonary hypertension, primary, 2

Diseases related to Primary Pulmonary Hypertension via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 457)
idRelated DiseaseScoreTop Affiliating Genes
1pulmonary disease, chronic obstructive30.9SLC6A4, NPPB, PDE5A
2mixed connective tissue disease30.9BMPR2, FBN1, ACVRL1
3thromboembolism30.7THBD, FGA
4systemic lupus erythematosus30.6FBN1, FGA, THBD, ALOX5
5eisenmenger syndrome30.6THBD
6aortic valve stenosis30.5BMP2, NPPA, NPPB
7hepatopulmonary syndrome30.4EDN1
8hereditary hemorrhagic telangiectasia30.4BMPR2, ACVRL1
9portal hypertension30.4EDN1
10insulin resistance30.3EDN1
11hyperhomocysteinemia30.3THBD, FBN1
12mitral valve stenosis30.2NPPB, NPPA, FGA
13systemic scleroderma30.2EDN1, FBN1, FGA, THBD, ALOX5
14dysfibrinogenemia30.1THBD, FGA
15conn's syndrome30.1ADM, NPPA
16sickle cell disease30.1FGA, EDN1
17arthritis30.1ALOX5, FBN1, BMP2
18thrombocytopenia30.1FGA, THBD, ALOX5
19vascular disease30.1PDE5A, BMPR2, EDN1, FGA, THBD, SLC6A4
20coronary artery disease,30.1PDE5A, NPPB, NPPA, EDN1, FBN1, FGA
21atherosclerosis30.1PDE5A, BMP2, EDN1, FBN1, FGA, THBD
22central sleep apnea30.1NPPB, KCNK3
23amyloidosis29.8NPPB
24fibrodysplasia ossificans progressiva29.8SMAD9, BMP2
25kawasaki disease29.8ADM, NPPB
26cor pulmonale29.8NPPB, NPPA, BMPR2, EDN1
27septic shock29.8ADM, THBD, FGA, NPPB
28adenocarcinoma29.8ADM, THBD, BMP2
29sepsis29.8NPPB, FGA, THBD, ALOX5, ADM
30acute myocardial infarction29.8NPPB, NPPA, EDN1, FGA, THBD, SLC6A4
31telangiectasis29.8ACVRL1
32hepatorenal syndrome29.8EDN1, NPPA
33phenylketonuria10.8
34hypoxia10.7
35familial hypertension10.7
36pulmonary function10.6
37congenital diaphragmatic hernia10.6
38pulmonary hypertension, neonatal10.6
39bronchopulmonary dysplasia10.5
40ventricular septal defect10.5
41alveolar capillary dysplasia10.5
42patent ductus arteriosus10.5
43pulmonary hypertension, primary, 210.5
44familial pulmonary arterial hypertension leucopenia and atrial septal defect10.5
45idiopathic pulmonary fibrosis10.4
46pulmonary sarcoidosis10.4
47scimitar syndrome10.4
48hyperphenylalaninemia10.4
49thalassemia10.4
50hyperuricemia, pulmonary hypertension, renal failure, and alkalosis10.3

Graphical network of the top 20 diseases related to Primary Pulmonary Hypertension:



Diseases related to primary pulmonary hypertension

Clinical Features for Primary Pulmonary Hypertension

Sources:
47OMIM
See all sources

Clinical features from OMIM:

265400,178600

Drugs & Therapeutics for Primary Pulmonary Hypertension

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Primary Pulmonary Hypertension

Drug clinical trials:

Search ClinicalTrials for Primary Pulmonary Hypertension

Search NIH Clinical Center for Primary Pulmonary Hypertension

Search CenterWatch for Primary Pulmonary Hypertension

Inferred drug relations via UMLS61/NDF-RT41:

Genetic Tests for Primary Pulmonary Hypertension

Sources:
22GTR
See all sources

Genetic tests related to Primary Pulmonary Hypertension:

id Genetic test Affiliating Genes
1 Primary Pulmonary Hypertension22

Anatomical Context for Primary Pulmonary Hypertension

Sources:
31LifeMap Discovery™, 33MalaCards
See all sources

MalaCards organs/tissues related to Primary Pulmonary Hypertension:

33
Whole blood, Brain, Heart, Smooth muscle, Liver, Lung, Thyroid, Monocytes, T cells, Endothelial

LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine

Cells/anatomical compartments in embryo or adult related to Primary Pulmonary Hypertension:
id TissueAnatomical CompartmentCell Relevance
1 LungPulmonary Capillary Plexus  Affected by disease

Animal Models for Primary Pulmonary Hypertension or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

Publications for Primary Pulmonary Hypertension

Sources:
51PubMed
See all sources

Articles related to Primary Pulmonary Hypertension:

(show top 50)    (show all 541)
idTitleAuthorsYear
1
Cesarean section and primary pulmonary hypertension: the role of intravenous dexmedetomidine. (19157850)
2009
2
Primary biliary cirrhosis complicated by polymyositis and pulmonary hypertension. (18379158)
2008
3
Conventional radiology and right heart catheterization in estimating primary pulmonary hypertension and pulmonary hypertension secondary to left-sided valvular disease. (18326896)
2008
4
Gene symbol: BMPR2. Disease: Pulmonary hypertension, primary. (18386374)
2008
5
Recovery of protein-losing enteropathy after living-donor lobar lung transplantation in primary pulmonary hypertension. (16563983)
2006
6
Survival with first-line bosentan in patients with primary pulmonary hypertension. (15684287)
2005
7
Systemic collateral supply in patients with chronic thromboembolic and primary pulmonary hypertension: assessment with multi-detector row helical CT angiography. (15703314)
2005
8
Efficacy and optimal dose of sildenafil in primary pulmonary hypertension. (15721505)
2005
9
Acute effects of sildenafil in patients with primary pulmonary hypertension receiving epoprostenol. (14972773)
2004
10
Clinical significance of brain natriuretic peptide in primary pulmonary hypertension. (14998614)
2004
11
Expression of human herpesvirus 8 in primary pulmonary hypertension. (13679525)
2003
12
Acute endothelin A receptor antagonism improves pulmonary and systemic haemodynamics in patients with pulmonary arterial hypertension that is primary or autoimmune and related to congenital heart disease. (12975426)
2003
13
Intravascular ultrasound of the elastic pulmonary arteries: a new approach for the evaluation of primary pulmonary hypertension. (12591838)
2003
14
Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. (12234951)
2002
15
Oxidative stress in patients with primary pulmonary hypertension. (12447471)
2002
16
Pulmonary artery pressure-flow relations after prostacyclin in primary pulmonary hypertension. (11818317)
2002
17
The prognostic role of the ECG in primary pulmonary hypertension. (11834666)
2002
18
Hyperplasia of pulmonary artery smooth muscle cells is causally related to overexpression of the serotonin transporter in primary pulmonary hypertension. (11893722)
2002
19
An unexpected case of primary pulmonary hypertension of the neonate (PPHN). Potential role of topical administration of enoxolone. (12442611)
2002
20
Plasminogen activation by blood monocytes and alveolar macrophages in primary pulmonary hypertension. (12138369)
2002
21
Gas exchange detection of exercise-induced right-to-left shunt in patients with primary pulmonary hypertension. (11772876)
2002
22
Electrocardiography to define clinical status in primary pulmonary hypertension and pulmonary arterial hypertension secondary to collagen vascular disease. (12171826)
2002
23
The enigma of primary pulmonary hypertension. (11300467)
2001
24
Pulmonary artery reflection for differentially diagnosing primary pulmonary hypertension and chronic pulmonary thromboembolism. (11451277)
2001
25
Exercise pathophysiology in patients with primary pulmonary hypertension. (11468205)
2001
26
Rapid regression of primary pulmonary hypertension. (11410578)
2001
27
Nocturnal hypoxemia is common in primary pulmonary hypertension. (11555526)
2001
28
Pregnancy and primary pulmonary hypertension : successful outcome with epoprostenol therapy. (11243988)
2001
29
Primary pulmonary hypertension. (11000779)
2000
30
Mortality from primary pulmonary hypertension in the United States, 1979-1996. (10713009)
2000
31
Twin pregnancy in a woman on long-term epoprostenol therapy for primary pulmonary hypertension. A case report. (10710749)
2000
32
Primary pulmonary hypertension and thyroid disease. (11035704)
2000
33
Pulmonary artery stenosis 5 years after single lung transplantation in primary pulmonary hypertension. (10612386)
1999
34
Effect of orally active prostacyclin analogue on survival of outpatients with primary pulmonary hypertension. (10520811)
1999
35
Primary pulmonary hypertension and anorectic drugs. (9988623)
1999
36
Familial primary pulmonary hypertension locus mapped to chromosome 2q31-q32. (9676630)
1998
37
Role of human immunodeficiency virus in primary pulmonary hypertension--case reports. (9855375)
1998
38
Vasodilators in the treatment of primary pulmonary hypertension. (9727771)
1998
39
Attenuated K+ channel gene transcription in primary pulmonary hypertension. (9504523)
1998
40
Noninvasive differential diagnosis between chronic pulmonary thromboembolism and primary pulmonary hypertension by means of Doppler ultrasound measurement. (9581735)
1998
41
Primary pulmonary hypertension in a patient with HIV infection. (10063433)
1998
42
Tumour microembolism presenting as "primary pulmonary hypertension". (9487355)
1997
43
HIV-associated primary pulmonary hypertension. A case control study. Swiss HIV Cohort Study. (9117037)
1997
44
Primary pulmonary hypertension: a continuing diagnostic and therapeutic challenge. (9151522)
1997
45
Intravenous epoprostenol for primary pulmonary hypertension. (8618596)
1996
46
Medical management of primary pulmonary hypertension. (8947091)
1996
47
Familial association of primary pulmonary hypertension and a new low-oxygen affinity beta-chain hemoglobinopathy, Hb Washtenaw. (8617104)
1996
48
Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. (7534664)
1995
49
Understanding right and left ventricular systolic function and interactions at rest and with exercise in primary pulmonary hypertension. (7856531)
1995
50
A patient with congenital plasminogen deficiency manifesting primary pulmonary hypertension. (8358126)
1993

Genetic Variations for Primary Pulmonary Hypertension

Expression for genes affiliated with Primary Pulmonary Hypertension

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Primary Pulmonary Hypertension

Search GEO for disease gene expression data for Primary Pulmonary Hypertension.

Pathways for genes affiliated with Primary Pulmonary Hypertension

Sources:
54Reactome, 52QIAGEN, 30KEGG, 38NCBI BioSystems Database, 4Cell Signaling Technology, 53R&D Systems, 56SinoBiological
See all sources

Pathways related to Primary Pulmonary Hypertension according to GeneCards/GeneDecks:

(show all 17)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.4THBD, FGA
210.3BMPR1B, BMP2, BMPR2
310.3BMPR1B, BMP2, BMPR2
410.3BMPR2, SMAD1, SMAD9
510.3SMAD9, SMAD1, BMPR2
610.3BMP2, BMPR2, SMAD1
710.3SMAD9, SMAD1, ACVRL1
810.3BMPR1B, BMP2, BMPR2, ACVRL1
9
Hide members
10.3ACVRL1, BMPR2, BMP2, BMPR1B
1010.2BMPR2, ACVRL1, SMAD1, SMAD9
11
Hide members
10.2BMPR1B, BMP2, BMPR2, SMAD1
1210.2SMAD1, ACVRL1, BMPR2, BMP2
13
Hide members
10.2BMP2, FBN1, FGA, THBD
1410.2BMPR2, BMP2, BMPR1B, NPPA, NPPB
15
Hide members
10.2NPPB, NPPA, BMPR1B, BMP2, BMPR2
1610.2SMAD9, SMAD1, BMPR2, BMP2, BMPR1B
17
Hide members
9.9BMPR1B, BMP2, BMPR2, EDN1, FBN1, FGA

Compounds for genes affiliated with Primary Pulmonary Hypertension

Sources:
45Novoseek, 29IUPHAR, 50PharmGKB, 60Tocris Bioscience, 11DrugBank, 24HMDB, 2BitterDB
See all sources

Compounds related to Primary Pulmonary Hypertension according to GeneCards/GeneDecks:

(show top 50)    (show all 121)
idCompoundScoreTop Affiliating Genes
1captopril45 29 50 60 1114.7ADM, EDN1
2norepinephrine45 11 2412.7ADM, SLC6A4, PDE5A
3estrogen4510.7PDE5A, SLC6A4, NPPB
46-ketoprostaglandin f1alpha4510.7NPPA, FGA, THBD, EDN1
5ptca4510.7NPPB, ADM, THBD, FGA
6urodilatin4510.7EDN1, NPPB, NPPA
78-bromo cgmp4510.6NPPB, PDE5A, NPPA, ADM
8phosphoramidon45 1111.6EDN1, NPPA, ADM, NPPB
9carvedilol45 50 29 1113.6ADM, NPPB, NPPA, EDN1
10omapatrilat4510.6NPPA, EDN1, ADM
11hanp4510.6NPPB, NPPA, EDN1
12spec-t4510.6NPPB, ADM, SLC6A4, NPPA
13isoproterenol45 1111.6ADM, PDE5A
14trandolapril45 1111.6NPPB, NPPA, EDN1
15losartan45 50 29 1113.6ADM, EDN1, NPPA, NPPB, THBD
16iloprost45 60 29 1113.6THBD, ADM, EDN1, PDE5A
17nitroglycerin45 1111.6NPPB, NPPA, EDN1, ADM, PDE5A
18sildenafil45 50 29 11 2414.6NPPA, NPPB, PDE5A, SLC6A4, EDN1
19bosentan45 1111.6ADM, NPPB, EDN1
20pge14510.6THBD, ADM, NPPA, FGA, PDE5A
21ang ii4510.6EDN1, ADM, NPPB, NPPA
22nitrate4510.6ADM, EDN1, NPPB, PDE5A
23txb24510.6ALOX5, THBD, EDN1, FGA
24oxygen45 2411.5BMPR2, FBN1, BMP2, PDE5A, ADM
25creatinine4510.5THBD, FGA, BMP2, NPPA, NPPB
26sodium nitroprusside4510.5EDN1, PDE5A, ADM, NPPB, NPPA
27l-nmma4510.5ADM, EDN1, NPPA, NPPB
28ibmx45 29 6012.5PDE5A, NPPA, BMP2, EDN1, THBD, ADM
29enalaprilat4510.5EDN1, NPPA, NPPB
30cgmp45 2911.5NPPA, EDN1, ADM, PDE5A, NPPB
31epinephrine45 11 2412.5NPPA, NPPB, EDN1, FGA, SLC6A4, ALOX5
32pentoxifylline45 1111.5NPPB, BMP2, PDE5A, THBD
33thromboxane45 2411.5ALOX5, FGA, EDN1, THBD
34histamine45 29 2412.4ADM, THBD, NPPB, ALOX5, PDE5A, EDN1
35indomethacin45 60 29 1113.4ALOX5, PDE5A, NPPB, BMP2, EDN1, ADM
36forskolin45 50 1112.4ADM, NPPA, NPPB, PDE5A
37phenylephrine45 29 11 2413.4NPPB, NPPA, EDN1, ADM
38sodium45 2411.4PDE5A, EDN1, THBD, NPPA, SLC6A4, ADM
39prostacyclin4510.4NPPB, NPPA, PDE5A, EDN1, FGA, THBD
40papaverine45 2 1112.4PDE5A, NPPB, EDN1
41mibg4510.4NPPB, NPPA, ADM
42nitric oxide45 11 2412.3BMP2, BMPR2, NPPB, PDE5A, NPPA, THBD
43vegf4510.3ALOX5, BMP2, NPPA, NPPB, PDE5A, THBD
44serine4510.3BMP2, SMAD1, ACVRL1, BMPR2, SLC6A4, FGA
45cyclosporin a45 29 6012.3NPPB, NPPA, BMP2, EDN1, ADM, THBD
46thromboxane a245 2411.2ADM, FGA, EDN1, ALOX5
47thiorphan45 29 1112.2NPPA, ADM, EDN1
48arginine4510.2ADM, KCNK3, PDE5A, NPPB, NPPA, BMP2
49thymidine45 2411.1BMPR2, BMP2, ADM, FGA, EDN1, NPPB
50cacl24510.0FBN1, FGA, THBD

GO Terms for genes affiliated with Primary Pulmonary Hypertension

Sources:
16Gene Ontology
See all sources

Cellular components related to Primary Pulmonary Hypertension according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1integral to plasma membraneGO:00588710.1ACVRL1, SLC6A4, THBD, BMPR2, BMPR1B, KCNK3
2cell surfaceGO:00998610.1BMP2, BMPR2, FGA, THBD, ACVRL1
3extracellular spaceGO:00561510.0ADM, NPPB, NPPA, BMP2, EDN1, FBN1
4extracellular regionGO:0055769.7NPPB, NPPA, BMP2, EDN1, FBN1, FGA

Biological processes related to Primary Pulmonary Hypertension according to GeneCards/GeneDecks:

(show all 33)
idNameGO IDScoreTop Affiliating Genes
1venous blood vessel developmentGO:06084110.7ACVRL1, BMPR2
2negative regulation of vasoconstrictionGO:04590610.7ADM, BMPR2
3lymphatic endothelial cell differentiationGO:06083610.7BMPR2, ACVRL1
4negative regulation of DNA biosynthetic processGO:200027910.7ACVRL1, BMPR2
5positive regulation of pathway-restricted SMAD protein phosphorylationGO:01086210.7BMP2, BMPR2, ACVRL1
6positive regulation of bone mineralizationGO:03050110.7BMPR2, BMP2, BMPR1B
7positive regulation of transcription from RNA polymerase II promoter involved in cellular response to chemical stimulusGO:190152210.7BMP2, SMAD1
8artery developmentGO:06084010.7ACVRL1, BMPR2
9positive regulation of odontogenesisGO:04248210.7BMP2, EDN1
10cellular response to BMP stimulusGO:07177310.7SMAD1, ACVRL1, BMP2
11retina vasculature development in camera-type eyeGO:06129810.7ACVRL1, BMPR2
12negative regulation of cell growthGO:03030810.6NPPB, NPPA, BMPR2, ACVRL1
13positive regulation of endothelial cell proliferationGO:00193810.6ACVRL1, BMPR2, BMP2
14positive regulation of osteoblast differentiationGO:04566910.6BMPR1B, BMP2, BMPR2, SMAD1
15cGMP biosynthetic processGO:00618210.6NPPA, NPPB
16regulation of blood pressureGO:00821710.6NPPB, NPPA, ACVRL1
17body fluid secretionGO:00758910.6EDN1, NPPB
18heart developmentGO:00750710.6BMP2, EDN1, FBN1, ADM
19cartilage developmentGO:05121610.6EDN1, SMAD1, SMAD9
20female pregnancyGO:00756510.6NPPA, THBD, ADM
21positive regulation of cardiac muscle hypertrophyGO:01061310.5EDN1, PDE5A
22response to hypoxiaGO:00166610.5BMP2, EDN1, SLC6A4, SMAD9, ADM
23protein phosphorylationGO:00646810.5BMPR1B, BMP2, ACVRL1, SMAD1, SMAD9
24positive regulation of cartilage developmentGO:06103610.5BMP2, SMAD1
25receptor guanylyl cyclase signaling pathwayGO:00716810.5NPPB, NPPA
26lymphangiogenesisGO:00194610.5BMPR2, ACVRL1
27BMP signaling pathwayGO:03050910.5SMAD9, SMAD1, ACVRL1, BMPR2, BMP2, BMPR1B
28positive regulation of urine volumeGO:03581010.5NPPB, EDN1
29positive regulation of heart rateGO:01046010.4EDN1, ADM
30negative regulation of blood coagulationGO:03019510.4THBD, EDN1
31vasoconstrictionGO:04231010.3SLC6A4, EDN1
32regulation of blood vessel sizeGO:05088010.3NPPB, NPPA
33positive regulation of renal sodium excretionGO:03581510.1NPPB, EDN1

Molecular functions related to Primary Pulmonary Hypertension according to GeneCards/GeneDecks:

(show all 9)
idNameGO IDScoreTop Affiliating Genes
1transforming growth factor beta receptor activity, type IGO:00502510.5ACVRL1, BMPR1B
2SMAD bindingGO:04633210.5BMPR1B, BMP2, ACVRL1
3hormone activityGO:00517910.4ADM, EDN1, NPPA, NPPB
4transforming growth factor beta receptor, pathway-specific cytoplasmic mediator activityGO:03061810.4SMAD9, SMAD1
5transforming growth factor beta-activated receptor activityGO:00502410.4ACVRL1, BMPR2
6receptor bindingGO:00510210.4NPPB, NPPA, BMP2, FGA, ADM
7transmembrane receptor protein serine/threonine kinase activityGO:00467510.3BMPR1B, ACVRL1
8receptor signaling protein serine/threonine kinase activityGO:00470210.1ACVRL1, BMPR1B
9protein bindingGO:0055159.7FBN1, EDN1, BMPR2, BMP2, BMPR1B, FGA

Products for genes affiliated with Primary Pulmonary Hypertension

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Primary Pulmonary Hypertension

3CDC
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet