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

A novel human fetal lung-derived alveolar organoid model reveals mechanisms of surfactant protein C maturation relevant to interstitial lung disease

一种新型的人类胎肺来源的肺泡类器官模型揭示了与间质性肺疾病相关的表面活性蛋白C成熟的机制。

Lim, Kyungtae; Rutherford, Eimear N; Delpiano, Livia; He, Peng; Lin, Weimin; Sun, Dawei; Van den Boomen, Dick J H; Edgar, James R; Bang, Jae Hak; Predeus, Alexander; Teichmann, Sarah A; Marioni, John C; Matesic, Lydia E; Lee, Joo-Hyeon; Lehner, Paul J; Marciniak, Stefan J; Rawlins, Emma L; Dickens, Jennifer A

Endogenous gene editing of alveolar organoids reveals that expression of pathogenic variant SFTPC-I73T disrupts endosomal function, epithelial polarity and wound healing.

肺泡类器官的内源基因编辑显示,致病变异体 SFTPC-I73T 的表达会破坏内体功能、上皮极性和伤口愈合

Rutherford Eimear N, Sun Dawei, Lim Kyungtae, Edgar James R, Matesic Lydia E, Marciniak Stefan J, Rawlins Emma L, Dickens Jennifer A

Brief Report: A Novel Sodium/Iodide Symporter Mutation, S356F, Causing Congenital Hypothyroidism

简报:一种新型钠/碘同向转运蛋白突变S356F导致先天性甲状腺功能减退症

Durgia, Harsh; Nicholas, Adeline K; Schoenmakers, Erik; Dickens, Jennifer A; Halanaik, Dhanapathi; Sahoo, Jayaprakash; Kamalanathan, Sadishkumar; Schoenmakers, Nadia

The endoplasmic reticulum remains functionally connected by vesicular transport after its fragmentation in cells expressing Z-α1-antitrypsin

在表达 Z-α1-抗胰蛋白酶的细胞中,内质网发生断裂后,仍通过囊泡运输保持功能性连接。

Dickens, Jennifer A; Ordóñez, Adriana; Chambers, Joseph E; Beckett, Alison J; Patel, Vruti; Malzer, Elke; Dominicus, Caia S; Bradley, Jayson; Peden, Andrew A; Prior, Ian A; Lomas, David A; Marciniak, Stefan J

A single-chain variable fragment intrabody prevents intracellular polymerization of Z α1-antitrypsin while allowing its antiproteinase activity

单链可变片段胞内抗体可阻止Zα1-抗胰蛋白酶的细胞内聚合,同时保留其抗蛋白酶活性。

Ordóñez, Adriana; Pérez, Juan; Tan, Lu; Dickens, Jennifer A; Motamedi-Shad, Neda; Irving, James A; Haq, Imran; Ekeowa, Ugo; Marciniak, Stefan J; Miranda, Elena; Lomas, David A

Altered native stability is the dominant basis for susceptibility of α1-antitrypsin mutants to polymerization

α1-抗胰蛋白酶突变体易发生聚合反应的主要原因是其天然稳定性改变。

Irving, James A; Haq, Imran; Dickens, Jennifer A; Faull, Sarah V; Lomas, David A

AB 96. Chemotherapy for lung cancer

AB 96. 肺癌化疗

Grouse, Lawrence; Haq, Imran; Irving, James A; Faull, Sarah V; Dickens, Jennifer A; Ordóñez, Adriana; Belorgey, Didier; Gooptu, Bibek; Lomas, David A; Ellis, Ronald; Riedmann, Eva M; Baliaka, Aggeliki; Iordanidis, Fotis; Tryfon, Stavros; Pastelli, Nikoleta; Sakkas, Leonidas; Nena, Evangelia; Kroustallaki, Eleftheria; Steiropoulos, Paschalis; Xanthoudaki, Maria; Vargemezis, Vassileios; Bouros, Demosthenes; Krasouli, Sotiria; Hromis, Sanja; Zvezdin, Biljana; Maksimovic, Olivera; Duric, Mirna; Sečen, Nevena; Bursać, Daliborka; Sazdanić-Velikić, Danica; Tepavac, Aleksandar; Šarčev, Tatjana

Why has it been so difficult to prove the efficacy of alpha-1-antitrypsin replacement therapy? Insights from the study of disease pathogenesis

为什么证明α1-抗胰蛋白酶替代疗法的疗效如此困难?来自疾病发病机制研究的启示

Dickens, Jennifer A; Lomas, David A

COPD association and repeatability of blood biomarkers in the ECLIPSE cohort

ECLIPSE队列中COPD与血液生物标志物的关联性和重复性

Dickens, Jennifer A; Miller, Bruce E; Edwards, Lisa D; Silverman, Edwin K; Lomas, David A; Tal-Singer, Ruth