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

Disruption of the human cystin-1 myristoyl-electrostatic switch causes polycystic kidney disease that phenocopies autosomal recessive polycystic kidney disease

人类胱氨酸-1肉豆蔻酰静电开关的破坏会导致多囊肾病,其表型与常染色体隐性多囊肾病相似。

Yang, Chaozhe; Harafuji, Naoe; Watts, Jacob A; Tao, Binli; Moran, Claire; Clements, Jenna; Price, Kalyn; Laucevicius, Anthony; Burrill, Natalie; Gebb, Juliana; Soni, Shelly; Oliver, Edward; Savla, Jill J; Christ, Lori; Moldenhauer, Julie; Hartung, Erum A; Didier, Ryne; Santani, Avni; Sandford, Richard N; Selkirk, Lisa; Radley, Jessica A; Mann, Kathy; Simonicova, Ingrid; Karl, Rudolfo; Kariat Ashraf, Arsila Palliyulla; Wachten, Dagmar; Wilson, Landon; Bebok, Zsuzsanna; Caldovic, Ljubica; Guay-Woodford, Lisa M

Differential regulation of MYC expression by PKHD1/Pkhd1 in human and mouse kidneys: phenotypic implications for recessive polycystic kidney disease

PKHD1/Pkhd1 在人类和小鼠肾脏中对 MYC 表达的差异调节:对隐性多囊肾病的表型影响

Naoe Harafuji #, Chaozhe Yang #, Maoqing Wu, Girija Thiruvengadam, Heather Gordish-Dressman, R Griffin Thompson, P Darwin Bell, Avi Z Rosenberg, Claudia Dafinger, Max C Liebau, Zsuzsanna Bebok, Ljubica Caldovic, Lisa M Guay-Woodford

Cystin is required for maintaining fibrocystin (FPC) levels and safeguarding proteome integrity in mouse renal epithelial cells: A mechanistic connection between the kidney defects in cpk mice and human ARPKD

胱氨酸是维持小鼠肾上皮细胞中纤维囊蛋白 (FPC) 水平和保护蛋白质组完整性所必需的:cpk 小鼠肾脏缺陷与人类 ARPKD 之间的机制联系

Yiming J Zhang, Chaozhe Yang, Wei Wang, Naoe Harafuji, Piotr Stasiak, P Darwin Bell, Ljubica Caldovic, Elizabeth Sztul, Lisa M Guay-Woodford, Zsuzsanna Bebok

Pkhd1cyli/cyli mice have altered renal Pkhd1 mRNA processing and hormonally sensitive liver disease

Pkhd1cyli/cyli 小鼠的肾脏 Pkhd1 mRNA 处理发生了改变,并患上了激素敏感性肝病

Chaozhe Yang, Naoe Harafuji, Ljubica Caldovic, Weiying Yu, Ravindra Boddu, Surajit Bhattacharya, Hayk Barseghyan, Heather Gordish-Dressman, Oded Foreman, Zsuzsa Bebok, Eva M Eicher, Lisa M Guay-Woodford

LPS decreases CFTR open probability and mucociliary transport through generation of reactive oxygen species

LPS 通过产生活性氧降低 CFTR 开放概率和粘膜纤毛运输

Do Yeon Cho, Shaoyan Zhang, Ahmed Lazrak, Daniel Skinner, Harrison M Thompson, Jessica Grayson, Purushotham Guroji, Saurabh Aggarwal, Zsuzsanna Bebok, Steven M Rowe, Sadis Matalon, Eric J Sorscher, Bradford A Woodworth

miR-200b downregulates CFTR during hypoxia in human lung epithelial cells

miR-200b 在人肺上皮细胞缺氧期间下调 CFTR

Sylwia Bartoszewska, Wojciech Kamysz, Bogdan Jakiela, Marek Sanak, Jarosław Króliczewski, Zsuzsa Bebok, Rafal Bartoszewski #, James F Collawn #

A synonymous codon change alters the drug sensitivity of ΔF508 cystic fibrosis transmembrane conductance regulator

同义密码子变化改变了 ΔF508 囊性纤维化跨膜传导调节剂的药物敏感性

Vedrana Bali, Ahmed Lazrak, Purushotham Guroji, Lianwu Fu, Sadis Matalon, Zsuzsanna Bebok

Mechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic Fibrosis

改善囊性纤维化最常见致病突变校正的机制方法

Vedrana Bali, Ahmed Lazrak, Purushotham Guroji, Sadis Matalon, Zsuzsanna Bebok

Limited ATF4 Expression in Degenerating Retinas with Ongoing ER Stress Promotes Photoreceptor Survival in a Mouse Model of Autosomal Dominant Retinitis Pigmentosa

在持续的内质网应激下,退化视网膜中的 ATF4 表达有限,可促进常染色体显性视网膜色素变性小鼠模型中的光感受器存活

Yogesh Bhootada, Pravallika Kotla, Sergei Zolotukhin, Oleg Gorbatyuk, Zsuzsanna Bebok, Mohammad Athar, Marina Gorbatyuk

Influenza virus M2 targets cystic fibrosis transmembrane conductance regulator for lysosomal degradation during viral infection

流感病毒 M2 靶向囊性纤维化跨膜传导调节器,以便在病毒感染期间进行溶酶体降解

James David Londino, Ahmed Lazrak, James W Noah, Saurabh Aggarwal, Vedrana Bali, Bradford A Woodworth, Zsuzsanna Bebok, Sadis Matalon