相关参考文献
注意:仅列出部分参考文献,下载原文获取全部文献信息。A new role for heat shock factor 27 in the pathophysiology of Clostridium difficile toxin B
Murali K. Yanda et al.
AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY (2020)
Fibrocystin Is Essential to Cellular Control of Adhesion and Epithelial Morphogenesis
Wolfgang H. Ziegler et al.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2020)
Emerging Cystic Fibrosis Transmembrane Conductance Regulator Modulators as New Drugs for Cystic Fibrosis: A Portrait of in Vitro Pharmacology and Clinical Translation
Drishti P. Ghelani et al.
ACS PHARMACOLOGY & TRANSLATIONAL SCIENCE (2020)
Transcytosis maintains CFTR apical polarity in the face of constitutive and mutation-induced basolateral missorting
Aurelien Bidaud-Meynard et al.
JOURNAL OF CELL SCIENCE (2019)
Pharmacological reversal of renal cysts from secretion to absorption suggests a potential therapeutic strategy for managing autosomal dominant polycystic kidney disease
Murali K. Yanda et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2019)
A potential strategy for reducing cysts in autosomal dominant polycystic kidney disease with a CFTR corrector
Murali K. Yanda et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2018)
A chemical compound inhibiting the Aha1-Hsp90 chaperone complex
Sandrine C. Stiegler et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2017)
Histone deacetylase 6 inhibition reduces cysts by decreasing cAMP and Ca2+ in knock-out mouse models of polycystic kidney disease
Murali K. Yanda et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2017)
Efficacy and safety of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: a pooled analysis
J. Stuart Elborn et al.
LANCET RESPIRATORY MEDICINE (2016)
Rescuing Trafficking Mutants of the ATP-binding Cassette Protein, ABCA4, with Small Molecule Correctors as a Treatment for Stargardt Eye Disease
Inna Sabirzhanova et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2015)
Combination of Correctors Rescue ΔF508-CFTR by Reducing Its Association with Hsp40 and Hsp27
Miqueias Lopes-Pacheco et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2015)
Pro-inflammatory cytokines downregulate Hsp27 and cause apoptosis of human retinal capillary endothelial cells
Rooban B. Nahomi et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2014)
VER-155008, a small molecule inhibitor of HSP70 with potent anti-cancer activity on lung cancer cell lines
Wei Wen et al.
EXPERIMENTAL BIOLOGY AND MEDICINE (2014)
Anoctamin 1 induces calcium-activated chloride secretion and proliferation of renal cyst-forming epithelial cells
Bjoern Buchholz et al.
KIDNEY INTERNATIONAL (2014)
Autosomal Recessive Polycystic Kidney Disease: A Hepatorenal Fibrocystic Disorder With Pleiotropic Effects
Erum A. Hartung et al.
PEDIATRICS (2014)
Molecular Chaperone Functions in Protein Folding and Proteostasis
Yujin E. Kim et al.
ANNUAL REVIEW OF BIOCHEMISTRY, VOL 82 (2013)
Inhibiting the HSP90 chaperone slows cyst growth in a mouse model of autosomal dominant polycystic kidney disease
Tamina Seeger-Nukpezah et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2013)
Physiology of Cholangiocytes
James H. Tabibian et al.
COMPREHENSIVE PHYSIOLOGY (2013)
Pancreatic and biliary secretion are both altered in cystic fibrosis pigs
Aliye Uc et al.
AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY (2012)
Modeling general proteostasis: proteome balance in health and disease
Daniela M. Roth et al.
CURRENT OPINION IN CELL BIOLOGY (2011)
LARGE POTENTIALS OF SMALL HEAT SHOCK PROTEINS
Evgeny V. Mymrikov et al.
PHYSIOLOGICAL REVIEWS (2011)
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
Fredrick Van Goor et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
The ΔF508 Mutation Causes CFTR Misprocessing and Cystic Fibrosis-Like Disease in Pigs
Lynda S. Ostedgaard et al.
SCIENCE TRANSLATIONAL MEDICINE (2011)
Emergent Properties of Proteostasis in Managing Cystic Fibrosis
William E. Balch et al.
COLD SPRING HARBOR PERSPECTIVES IN BIOLOGY (2011)
HSPB1, actin filament dynamics, and aging cells
Bindi M. Doshi et al.
AGING, CANCER, AND AGE-RELATED DISEASES: COMMON MECHANISM? (2010)
Versatility of the small heat shock protein HSPB6 (Hsp20)
Alim S. Seit-Nebi et al.
CELL STRESS & CHAPERONES (2010)
New Molecular and Biological Mechanism of Antitumor Activities of KW-2478, a Novel Nonansamycin Heat Shock Protein 90 Inhibitor, in Multiple Myeloma Cells
Takayuki Nakashima et al.
CLINICAL CANCER RESEARCH (2010)
HSP90 at the hub of protein homeostasis: emerging mechanistic insights
Mikko Taipale et al.
NATURE REVIEWS MOLECULAR CELL BIOLOGY (2010)
Therapeutic Potential of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Inhibitors in Polycystic Kidney Disease
Hongyu Li et al.
BIODRUGS (2009)
Nanomolar Potency Pyrimido-pyrrolo-quinoxatinedione CFTR Inhibitor Reduces Cyst Size in a Polycystic Kidney Disease Model
Lukmanee Tradtrantip et al.
JOURNAL OF MEDICINAL CHEMISTRY (2009)
Biliary and pancreatic dysgenesis in mice harboring a mutation in Pkhd1
Anna-Rachel Gallagher et al.
AMERICAN JOURNAL OF PATHOLOGY (2008)
Small-molecule CFTR inhibitors slow cyst growth in polycystic kidney disease
Baoxue Yang et al.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY (2008)
Genetic interaction studies link autosomal dominant and recessive polycystic kidney disease in a common pathway
Miguel A. Garcia-Gonzalez et al.
HUMAN MOLECULAR GENETICS (2007)
The protective and destructive roles played by molecular chaperones during ERAD (endoplasmic-reticulum-associated degradation)
Jeffrey L. Brodsky
BIOCHEMICAL JOURNAL (2007)
Hsp27 knockdown using nucleotide-based therapies inhibit tumor growth and enhance chemotherapy in human bladder cancer cells
Masayuki Kamada et al.
MOLECULAR CANCER THERAPEUTICS (2007)
Molecular and cellular pathophysiology of autosomal recessive polycystic kidney disease (ARPKD)
William E. Sweeney et al.
CELL AND TISSUE RESEARCH (2006)
Liver disease in cystic fibrosis
Carla Colombo et al.
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION (2006)
CFTR chloride channel drug discovery - Inhibitors as antidiarrheals and activators for therapy of cystic fibrosis
A. S. Verkman et al.
CURRENT PHARMACEUTICAL DESIGN (2006)
Inhibition of Pkhd1 impairs tubulomorphogenesis of cultured IMCD cells
WY Mai et al.
MOLECULAR BIOLOGY OF THE CELL (2005)
Molecular genetics of autosomal recessive polycystic kidney disease
PC Harris et al.
MOLECULAR GENETICS AND METABOLISM (2004)
Heat shock protein 70 promotes cancer cell viability by safeguarding lysosomal integrity
M Gyrd-Hansen et al.
CELL CYCLE (2004)
Polyductin, the PKHD1 gene product, comprises isoforms expressed in plasma membrane, primary cilium, and cytoplasm
LFC Menezes et al.
KIDNEY INTERNATIONAL (2004)
Identification and characterization of Pkhd1, the mouse orthologue of the human ARPKD gene
Y Nagasawa et al.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY (2002)
PKHD1, the polycystic kidney and hepatic disease 1 gene, encodes a novel large protein containing multiple immunoglobulin-like plexin-transcription-factor domains and parallel beta-helix 1 repeats
LF Onuchic et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2002)
The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein
CJ Ward et al.
NATURE GENETICS (2002)
Defects in processing and trafficking of the cystic fibrosis transmembrane conductance regulator
WR Skach
KIDNEY INTERNATIONAL (2000)