(Q28534831)

heritable pulmonary arterial hypertension

inferred from title

1 reference

Andrea L Frump

1

0 references

Jonathan W Lowery

2

0 references

Rizwan Hamid

3

0 references

Eric D Austin

4

0 references

Mark de Caestecker

Creative Commons Attribution 4.0 International

5

0 references

language of work or name

English

0 references

publication date

2013

0 references

published in

PLOS One

0 references

volume

8

0 references

issue

11

0 references

page(s)

e80319

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copyright license

start time

5 November 2013

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

0 references

cites work

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Bmp2 and Bmp4 exert opposing effects in hypoxic pulmonary hypertension

19 March 2017

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Inflammation, endothelial injury, and persistent pulmonary hypertension in heterozygous BMPR2-mutant mice

19 March 2017

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The BMP type II receptor is located in lipid rafts, including caveolae, of pulmonary endothelium in vivo and in vitro

19 March 2017

1 reference

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BMP signaling is required for septation of the outflow tract of the mammalian heart

19 March 2017

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Functional analysis of bone morphogenetic protein type II receptor mutations underlying primary pulmonary hypertension

19 March 2017

1 reference

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Correction of nonsense BMPR2 and SMAD9 mutations by ataluren in pulmonary arterial hypertension.

19 March 2017

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A host-guest relationship in bone morphogenetic protein receptor-II defines specificity in ligand-receptor recognition

29 September 2017

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Promiscuity and specificity in BMP receptor activation

29 September 2017

1 reference

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Idiopathic and heritable PAH perturb common molecular pathways, correlated with increased MSX1 expression.

29 September 2017

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Defective trafficking of cone photoreceptor CNG channels induces the unfolded protein response and ER-stress-associated cell death

29 September 2017

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Pulmonary arterial hypertension: insights from genetic studies

29 September 2017

1 reference

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The chemical chaperones tauroursodeoxycholic and 4-phenylbutyric acid accelerate thyroid hormone activation and energy expenditure

29 September 2017

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ID family protein expression and regulation in hypoxic pulmonary hypertension

29 September 2017

1 reference

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29 September 2017

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Transcripts from a novel BMPR2 termination mutation escape nonsense mediated decay by downstream translation re-initiation: implications for treating pulmonary hypertension

29 September 2017

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29 September 2017

1 reference

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Tauroursodeoxycholic acid attenuates lipid accumulation in endoplasmic reticulum-stressed macrophages

29 September 2017

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Truncating and missense BMPR2 mutations differentially affect the severity of heritable pulmonary arterial hypertension

29 September 2017

1 reference

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29 September 2017

1 reference

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Penetrance of pulmonary arterial hypertension is modulated by the expression of normal BMPR2 allele

29 September 2017

1 reference

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Gene expression in BMPR2 mutation carriers with and without evidence of pulmonary arterial hypertension suggests pathways relevant to disease penetrance

29 September 2017

1 reference

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Proinflammatory effect of sodium 4-phenylbutyrate in deltaF508-cystic fibrosis transmembrane conductance regulator lung epithelial cells: involvement of extracellular signal-regulated protein kinase 1/2 and c-Jun-NH2-terminal kinase signaling

29 September 2017

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The glycan code of the endoplasmic reticulum: asparagine-linked carbohydrates as protein maturation and quality-control tags

29 September 2017

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Id: a target of BMP signaling

29 September 2017

1 reference

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Functional heterogeneity of bone morphogenetic protein receptor-II mutants found in patients with primary pulmonary hypertension

29 September 2017

1 reference

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Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate

29 September 2017

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BMPR2 haploinsufficiency as the inherited molecular mechanism for primary pulmonary hypertension

29 September 2017

1 reference

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Establishment of conditionally immortalized epithelial cell lines from both colon and small intestine of adult H-2Kb-tsA58 transgenic mice

29 September 2017

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Transport of secretory and membrane glycoproteins from the rough endoplasmic reticulum to the Golgi. A rate-limiting step in protein maturation and secretion.

29 September 2017

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Chloroquine prevents progression of experimental pulmonary hypertension via inhibition of autophagy and lysosomal bone morphogenetic protein type II receptor degradation

29 September 2017

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Functional rescue of mutant ABCA1 proteins by sodium 4-phenylbutyrate

2 June 2018

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Doxycycline plus tauroursodeoxycholic acid for transthyretin amyloidosis: a phase II study

2 June 2018

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Genetic ablation of the BMPR2 gene in pulmonary endothelium is sufficient to predispose to pulmonary arterial hypertension.

2 June 2018

1 reference

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Failure of bone morphogenetic protein receptor trafficking in pulmonary arterial hypertension: potential for rescue

2 June 2018

1 reference

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Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension

2 June 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

Serotonin increases susceptibility to pulmonary hypertension in BMPR2-deficient mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

BMPR-II heterozygous mice have mild pulmonary hypertension and an impaired pulmonary vascular remodeling response to prolonged hypoxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

Bone morphogenetic protein receptor II regulates pulmonary artery endothelial cell barrier function

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

Dosage-dependent requirement of BMP type II receptor for maintenance of vascular integrity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

Modulation of epithelial sodium channel trafficking and function by sodium 4-phenylbutyrate in human nasal epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

Modulation of deltaF508 cystic fibrosis transmembrane regulator trafficking and function with 4-phenylbutyrate and flavonoids

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

Bone morphogenetic protein 4 promotes pulmonary vascular remodeling in hypoxic pulmonary hypertension.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

High frequency of BMPR2 exonic deletions/duplications in familial pulmonary arterial hypertension

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

Endoglycosidase and glycoamidase release of N-linked glycans.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

Primary pulmonary hypertension is associated with reduced pulmonary vascular expression of type II bone morphogenetic protein receptor

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

Characterization of ligand-binding properties of the human BMP type II receptor extracellular domain

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0080319

Increased susceptibility to hypoxic pulmonary hypertension in Bmpr2 mutant mice is associated with endothelial dysfunction in the pulmonary vasculature

21 January 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/24224048

12 December 2020

inferred from PubMed ID database lookup

A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/24224048

12 December 2020

inferred from PubMed ID database lookup

Gross BMPR2 gene rearrangements constitute a new cause for primary pulmonary hypertension

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/24224048

12 December 2020

inferred from PubMed ID database lookup

Identifiers

DOI

10.1371/JOURNAL.PONE.0080319

1 reference

ADS bibcode

2013PLoSO...880319F

0 references

1 reference

1 reference

PubMed publication ID

24224048

1 reference