Detalhe da pesquisa
1.
EMILIN1 deficiency causes arterial tortuosity with osteopenia and connects impaired elastogenesis with defective collagen fibrillogenesis.
Am J Hum Genet
; 109(12): 2230-2252, 2022 12 01.
Artigo
Inglês
| MEDLINE | ID: mdl-36351433
2.
Pathogenic variants in THSD4, encoding the ADAMTS-like 6 protein, predispose to inherited thoracic aortic aneurysm.
Genet Med
; 23(1): 111-122, 2021 01.
Artigo
Inglês
| MEDLINE | ID: mdl-32855533
3.
The acute kidney injury to chronic kidney disease transition in a mouse model of acute cardiorenal syndrome emphasizes the role of inflammation.
Kidney Int
; 97(1): 95-105, 2020 01.
Artigo
Inglês
| MEDLINE | ID: mdl-31623859
4.
Intracellular mechanisms of molecular recognition and sorting for transport of large extracellular matrix molecules.
Proc Natl Acad Sci U S A
; 113(41): E6036-E6044, 2016 10 11.
Artigo
Inglês
| MEDLINE | ID: mdl-27679847
5.
Codependence of Bone Morphogenetic Protein Receptor 2 and Transforming Growth Factor-ß in Elastic Fiber Assembly and Its Perturbation in Pulmonary Arterial Hypertension.
Arterioscler Thromb Vasc Biol
; 37(8): 1559-1569, 2017 08.
Artigo
Inglês
| MEDLINE | ID: mdl-28619995
6.
Abnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null Mice.
PLoS Genet
; 11(6): e1005340, 2015 Jun.
Artigo
Inglês
| MEDLINE | ID: mdl-26114882
7.
Latent TGF-ß binding protein 4 promotes elastic fiber assembly by interacting with fibulin-5.
Proc Natl Acad Sci U S A
; 110(8): 2852-7, 2013 Feb 19.
Artigo
Inglês
| MEDLINE | ID: mdl-23382201
8.
Corneal stroma microfibrils.
Exp Eye Res
; 132: 198-207, 2015 Mar.
Artigo
Inglês
| MEDLINE | ID: mdl-25613072
9.
Thoracic aortic aneurysm frequency and dissection are associated with fibrillin-1 fragment concentrations in circulation.
Circ Res
; 113(10): 1159-68, 2013 Oct 25.
Artigo
Inglês
| MEDLINE | ID: mdl-24036495
10.
Microenvironmental regulation by fibrillin-1.
PLoS Genet
; 8(1): e1002425, 2012 Jan.
Artigo
Inglês
| MEDLINE | ID: mdl-22242013
11.
Homotypic versican G1 domain interactions enhance hyaluronan incorporation into fibrillin microfibrils.
J Biol Chem
; 288(40): 29170-81, 2013 Oct 04.
Artigo
Inglês
| MEDLINE | ID: mdl-23963449
12.
Early fibrillin-1 assembly monitored through a modifiable recombinant cell approach.
Biomacromolecules
; 15(4): 1456-68, 2014 Apr 14.
Artigo
Inglês
| MEDLINE | ID: mdl-24559401
13.
Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome.
Nat Genet
; 33(3): 407-11, 2003 Mar.
Artigo
Inglês
| MEDLINE | ID: mdl-12598898
14.
Unraveling the role of TGFß signaling in thoracic aortic aneurysm and dissection using Fbn1 mutant mouse models.
Matrix Biol
; 123: 17-33, 2023 Nov.
Artigo
Inglês
| MEDLINE | ID: mdl-37683955
15.
Prodomains of transforming growth factor beta (TGFbeta) superfamily members specify different functions: extracellular matrix interactions and growth factor bioavailability.
J Biol Chem
; 286(7): 5087-99, 2011 Feb 18.
Artigo
Inglês
| MEDLINE | ID: mdl-21135108
16.
ADAMTS10 protein interacts with fibrillin-1 and promotes its deposition in extracellular matrix of cultured fibroblasts.
J Biol Chem
; 286(19): 17156-67, 2011 May 13.
Artigo
Inglês
| MEDLINE | ID: mdl-21402694
17.
Specificity of latent TGF-ß binding protein (LTBP) incorporation into matrix: role of fibrillins and fibronectin.
J Cell Physiol
; 227(12): 3828-36, 2012 Dec.
Artigo
Inglês
| MEDLINE | ID: mdl-22495824
18.
Mutations in LTBP4 cause a syndrome of impaired pulmonary, gastrointestinal, genitourinary, musculoskeletal, and dermal development.
Am J Hum Genet
; 85(5): 593-605, 2009 Nov.
Artigo
Inglês
| MEDLINE | ID: mdl-19836010
19.
A Novel Model of Tobacco Smoke-Mediated Aortic Injury.
Vasc Endovascular Surg
; 56(3): 244-252, 2022 Apr.
Artigo
Inglês
| MEDLINE | ID: mdl-34961389
20.
Circulating fibrillin fragment concentrations in patients with and without aortic pathology.
JVS Vasc Sci
; 3: 389-402, 2022.
Artigo
Inglês
| MEDLINE | ID: mdl-36568280