Mutations of the TGF-β type II receptorBMPR2 in pulmonary arterial hypertension

TGF-β SIGNAL TRANSDUCTION

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Primary Pulmonary Hypertension

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2004 • 1,915 citations

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

2000 • 1,445 citations

Familial Primary Pulmonary Hypertension (Gene PPH1) Is Caused by Mutations in the Bone Morphogenetic Protein Receptor–II Gene

2000 • 1,184 citations

Distinct endocytic pathways regulate TGF-β receptor signalling and turnover

2003 • 1,115 citations

Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family

2000 • 725 citations

Clinical and Molecular Genetic Features of Pulmonary Hypertension in Patients with Hereditary Hemorrhagic Telangiectasia

2001 • 714 citations

Primary Pulmonary Hypertension Is Associated With Reduced Pulmonary Vascular Expression of Type II Bone Morphogenetic Protein Receptor

2002 • 653 citations

Atomic structure of the MAP kinase ERK2 at 2.3 Å resolution

1994 • 591 citations

BMPR2 Haploinsufficiency as the Inherited Molecular Mechanism for Primary Pulmonary Hypertension

2001 • 536 citations

Altered Growth Responses of Pulmonary Artery Smooth Muscle Cells From Patients With Primary Pulmonary Hypertension to Transforming Growth Factor-β ₁ and Bone Morphogenetic Proteins

2001 • 453 citations

BMP Type II Receptor Is Required for Gastrulation and Early Development of Mouse Embryos

2000 • 413 citations

Mutation in the Gene for Bone Morphogenetic Protein Receptor II as a Cause of Primary Pulmonary Hypertension in a Large Kindred

2001 • 366 citations

Dysfunctional Smad Signaling Contributes to Abnormal Smooth Muscle Cell Proliferation in Familial Pulmonary Arterial Hypertension

2005 • 355 citations

Molecular and functional analysis identifies ALK-1 as the predominant cause of pulmonary hypertension related to hereditary haemorrhagic telangiectasia

2003 • 339 citations

Direct signaling by the BMP type II receptor via the cytoskeletal regulator LIMK1

2003 • 319 citations

Microsatellite Instability of Endothelial Cell Growth and Apoptosis Genes Within Plexiform Lesions in Primary Pulmonary Hypertension

2001 • 271 citations

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

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Pulmonary Hypertension in Transgenic Mice Expressing a Dominant-Negative BMPRII Gene in Smooth Muscle

2004 • 260 citations

BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease

2004 • 256 citations

Transforming Growth Factor-β Receptor Mutations and Pulmonary Arterial Hypertension in Childhood

2005 • 221 citations

BMPR2 gene rearrangements account for a significant proportion of mutations in familial and idiopathic pulmonary arterial hypertension

2006 • 214 citations

BMPR2 germline mutations in pulmonary hypertension associated with fenfluramine derivatives

2002 • 207 citations

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

2002 • 205 citations

Increased Susceptibility to Pulmonary Hypertension in Heterozygous BMPR2-Mutant Mice

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Pulmonary Veno-occlusive Disease Caused by an Inherited Mutation in Bone Morphogenetic Protein Receptor II

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Histopathology of Primary Pulmonary Hypertension

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Distinct RB1 gene mutations with low penetrance in hereditary retinoblastoma

1994 • 140 citations

Functional Heterogeneity of Bone Morphogenetic Protein Receptor-II Mutants Found in Patients with Primary Pulmonary Hypertension

2002 • 131 citations

Functional interaction between BMPR-II and Tctex-1, a light chain of Dynein, is isoform-specific and disrupted by mutations underlying primary pulmonary hypertension

2003 • 129 citations

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

2005 • 118 citations

Investigation of Second Genetic Hits at the BMPR2 Locus as a Modulator of Disease Progression in Familial Pulmonary Arterial Hypertension

2005 • 87 citations

BMPR2 mutations found in Japanese patients with familial and sporadic primary pulmonary hypertension

2004 • 80 citations

Low frequency of BMPR2 mutations in a German cohort of patients with sporadic idiopathic pulmonary arterial hypertension

2004 • 73 citations

BMPR2 mutations have short lifetime expectancy in primary pulmonary hypertension

2005 • 32 citations

Primary pulmonary hypertension after amfepramone (diethylpropion) with BMPR2 mutation

2003 • 31 citations

Primary pulmonary hypertension may be a heterogeneous disease with a second locus on chromosome 2q31

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Coancestry in Apparently Sporadic Primary Pulmonary Hypertension

1995 • 25 citations

Bone morphogenetic protein receptor-II mutation Arg491Trp causes malignant phenotype of familial primary pulmonary hypertension

2004 • 13 citations

Novel Insertional Mutation in the Bone Morphogenetic Protein Receptor Type II Associated With Sporadic Primary Pulmonary Hypertension

2004 • 12 citations

Novel nonsense mutation of the BMPR‐II gene in a Japanese patient with familial primary pulmonary hypertension

2002 • 8 citations

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2004 • 6 citations

Identification of a novel mutation in the gene for bone morphogenetic protein receptor II in an Israeli patient with familial primary pulmonary hypertension.

2004 • 5 citations