日期:
2020 年 — 2026 年
2020
2021
2022
2023
2024
2025
2026
影响因子:

Fibrillin microfibril structure identifies long-range effects of inherited pathogenic mutations affecting a key regulatory latent TGFβ-binding site

纤维蛋白微纤维结构可识别影响关键调节性潜在 TGFβ 结合位点的遗传致病突变的长期影响

Alan R F Godwin, Rana Dajani, Xinyang Zhang, Jennifer Thomson, David F Holmes, Christin S Adamo, Gerhard Sengle, Michael J Sherratt, Alan M Roseman, Clair Baldock

Skin fibroblasts of patients with geleophysic dysplasia due to FBN1 mutations have lysosomal inclusions and losartan improves their microfibril deposition defect

FBN1 突变导致皮肤组织发育不良患者的皮肤成纤维细胞含有溶酶体内含物,氯沙坦可改善其微纤维沉积缺陷

Pasquale Piccolo, Valeria Sabatino, Pratibha Mithbaokar, Elena Polishchuk, John Hicks, Roman Polishchuk, Carlos A Bacino, Nicola Brunetti-Pierri

A disintegrin-like and metalloprotease domain containing thrombospondin type 1 motif-like 5 (ADAMTSL5) is a novel fibrillin-1-, fibrillin-2-, and heparin-binding member of the ADAMTS superfamily containing a netrin-like module

含有血小板反应蛋白 1 型基序样 5 (ADAMTSL5) 的解整合素样和金属蛋白酶结构域是 ADAMTS 超家族中含有 netrin 样模块的新型原纤维蛋白-1、原纤维蛋白-2 和肝素结合成员

Hannah L Bader, Lauren W Wang, Jason C Ho, Thu Tran, Paul Holden, Jamie Fitzgerald, Radhika P Atit, Dieter P Reinhardt, Suneel S Apte

ADAMTS10 protein interacts with fibrillin-1 and promotes its deposition in extracellular matrix of cultured fibroblasts

ADAMTS10 蛋白与原纤维蛋白-1 相互作用并促进其在培养成纤维细胞的细胞外基质中沉积

Wendy E Kutz, Lauren W Wang, Hannah L Bader, Alana K Majors, Kazushi Iwata, Elias I Traboulsi, Lynn Y Sakai, Douglas R Keene, Suneel S Apte