376 related articles for article (PubMed ID: 25979247)
1. A microfibril assembly assay identifies different mechanisms of dominance underlying Marfan syndrome, stiff skin syndrome and acromelic dysplasias.
Jensen SA; Iqbal S; Bulsiewicz A; Handford PA
Hum Mol Genet; 2015 Aug; 24(15):4454-63. PubMed ID: 25979247
[TBL] [Abstract][Full Text] [Related]
2. New insights into the structure, assembly and biological roles of 10-12 nm connective tissue microfibrils from fibrillin-1 studies.
Jensen SA; Handford PA
Biochem J; 2016 Apr; 473(7):827-38. PubMed ID: 27026396
[TBL] [Abstract][Full Text] [Related]
3. Fibrillin protein pleiotropy: Acromelic dysplasias.
Sakai LY; Keene DR
Matrix Biol; 2019 Jul; 80():6-13. PubMed ID: 30219651
[TBL] [Abstract][Full Text] [Related]
4. Mutations in the TGFβ binding-protein-like domain 5 of FBN1 are responsible for acromicric and geleophysic dysplasias.
Le Goff C; Mahaut C; Wang LW; Allali S; Abhyankar A; Jensen S; Zylberberg L; Collod-Beroud G; Bonnet D; Alanay Y; Brady AF; Cordier MP; Devriendt K; Genevieve D; Kiper PÖ; Kitoh H; Krakow D; Lynch SA; Le Merrer M; Mégarbane A; Mortier G; Odent S; Polak M; Rohrbach M; Sillence D; Stolte-Dijkstra I; Superti-Furga A; Rimoin DL; Topouchian V; Unger S; Zabel B; Bole-Feysot C; Nitschke P; Handford P; Casanova JL; Boileau C; Apte SS; Munnich A; Cormier-Daire V
Am J Hum Genet; 2011 Jul; 89(1):7-14. PubMed ID: 21683322
[TBL] [Abstract][Full Text] [Related]
5. The fibrillin microfibril scaffold: A niche for growth factors and mechanosensation?
Sengle G; Sakai LY
Matrix Biol; 2015 Sep; 47():3-12. PubMed ID: 25957947
[TBL] [Abstract][Full Text] [Related]
6. Microenvironmental regulation by fibrillin-1.
Sengle G; Tsutsui K; Keene DR; Tufa SF; Carlson EJ; Charbonneau NL; Ono RN; Sasaki T; Wirtz MK; Samples JR; Fessler LI; Fessler JH; Sekiguchi K; Hayflick SJ; Sakai LY
PLoS Genet; 2012 Jan; 8(1):e1002425. PubMed ID: 22242013
[TBL] [Abstract][Full Text] [Related]
7. Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia--a novel mouse model providing insights into geleophysic dysplasia.
Hubmacher D; Wang LW; Mecham RP; Reinhardt DP; Apte SS
Dis Model Mech; 2015 May; 8(5):487-99. PubMed ID: 25762570
[TBL] [Abstract][Full Text] [Related]
8. Microfibrils: a cornerstone of extracellular matrix and a key to understand Marfan syndrome.
Bonetti MI
Ital J Anat Embryol; 2009; 114(4):201-24. PubMed ID: 20578676
[TBL] [Abstract][Full Text] [Related]
9. Acromelic dysplasias: how rare musculoskeletal disorders reveal biological functions of extracellular matrix proteins.
Stanley S; Balic Z; Hubmacher D
Ann N Y Acad Sci; 2021 Apr; 1490(1):57-76. PubMed ID: 32880985
[TBL] [Abstract][Full Text] [Related]
10. Assembly assay identifies a critical region of human fibrillin-1 required for 10-12 nm diameter microfibril biogenesis.
Jensen SA; Atwa O; Handford PA
PLoS One; 2021; 16(3):e0248532. PubMed ID: 33735269
[TBL] [Abstract][Full Text] [Related]
11. Fibrillin in Marfan syndrome and tight skin mice provides new insights into transforming growth factor-beta regulation and systemic sclerosis.
Lemaire R; Bayle J; Lafyatis R
Curr Opin Rheumatol; 2006 Nov; 18(6):582-7. PubMed ID: 17053502
[TBL] [Abstract][Full Text] [Related]
12. Exogenous activation of BMP-2 signaling overcomes TGFβ-mediated inhibition of osteogenesis in Marfan embryonic stem cells and Marfan patient-specific induced pluripotent stem cells.
Quarto N; Li S; Renda A; Longaker MT
Stem Cells; 2012 Dec; 30(12):2709-19. PubMed ID: 23037987
[TBL] [Abstract][Full Text] [Related]
13. Mutations in the human gene for fibrillin-1 (FBN1) in the Marfan syndrome and related disorders.
Dietz HC; Pyeritz RE
Hum Mol Genet; 1995; 4 Spec No():1799-809. PubMed ID: 8541880
[TBL] [Abstract][Full Text] [Related]
14. Engineered mutations in fibrillin-1 leading to Marfan syndrome act at the protein, cellular and organismal levels.
Zeyer KA; Reinhardt DP
Mutat Res Rev Mutat Res; 2015; 765():7-18. PubMed ID: 26281765
[TBL] [Abstract][Full Text] [Related]
15. Fibrillin-1 mutations in Marfan syndrome and other type-1 fibrillinopathies.
Hayward C; Brock DJ
Hum Mutat; 1997; 10(6):415-23. PubMed ID: 9401003
[TBL] [Abstract][Full Text] [Related]
16. Recent progress towards a molecular understanding of Marfan syndrome.
Dietz HC; Loeys B; Carta L; Ramirez F
Am J Med Genet C Semin Med Genet; 2005 Nov; 139C(1):4-9. PubMed ID: 16273535
[TBL] [Abstract][Full Text] [Related]
17. In vivo studies of mutant fibrillin-1 microfibrils.
Charbonneau NL; Carlson EJ; Tufa S; Sengle G; Manalo EC; Carlberg VM; Ramirez F; Keene DR; Sakai LY
J Biol Chem; 2010 Aug; 285(32):24943-55. PubMed ID: 20529844
[TBL] [Abstract][Full Text] [Related]
18. FBN1: The disease-causing gene for Marfan syndrome and other genetic disorders.
Sakai LY; Keene DR; Renard M; De Backer J
Gene; 2016 Oct; 591(1):279-291. PubMed ID: 27437668
[TBL] [Abstract][Full Text] [Related]
19. ADAMTSL6β protein rescues fibrillin-1 microfibril disorder in a Marfan syndrome mouse model through the promotion of fibrillin-1 assembly.
Saito M; Kurokawa M; Oda M; Oshima M; Tsutsui K; Kosaka K; Nakao K; Ogawa M; Manabe RI; Suda N; Ganjargal G; Hada Y; Noguchi T; Teranaka T; Sekiguchi K; Yoneda T; Tsuji T
J Biol Chem; 2011 Nov; 286(44):38602-38613. PubMed ID: 21880733
[TBL] [Abstract][Full Text] [Related]
20. Nanoscale Structural Comparison of Fibrillin-1 Microfibrils Isolated from Marfan and Non-Marfan Syndrome Human Aorta.
Șulea CM; Mártonfalvi Z; Csányi C; Haluszka D; Pólos M; Ágg B; Stengl R; Benke K; Szabolcs Z; Kellermayer MSZ
Int J Mol Sci; 2023 Apr; 24(8):. PubMed ID: 37108724
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
