ReviewPflugers Archiv : European journal of physiology2009
CFTR and defective endocytosis: new insights in the renal phenotype of cystic fibrosis.
Review in Pflugers Archiv : European journal of physiology, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
20 citing papers in PubMed, 41 citations in OpenAlex.
- Cystic fibrosis-related kidney disease-emerging morbidity and disease modifier.Pediatric nephrology (Berlin, Germany) · 2025Review
- Endocytosis at the maternal-fetal interface: balancing nutrient transport and pathogen defense.Frontiers in immunology · 2024Review
- Mechanisms by which the cystic fibrosis transmembrane conductance regulator may influence SARS-CoV-2 infection and COVID-19 disease severity.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2023Article
- Ion channels as a therapeutic target for renal fibrosis.Frontiers in physiology · 2022Review
- Urinary Exosomes of Patients with Cystic Fibrosis Unravel CFTR-Related Renal Disease.International journal of molecular sciences · 2020Article
- [Abdominal manifestations in cystic fibrosis : Clinical review].Der Radiologe · 2020Review
- Diabetic rats present higher urinary loss of proteins and lower renal expression of megalin, cubilin, ClC-5, and CFTR.Physiological reports · 2017Article
- Chloride transporters and receptor-mediated endocytosis in the renal proximal tubule.The Journal of physiology · 2015Review
- In-depth phenotyping of a Donnai-Barrow patient helps clarify proximal tubule dysfunction.Pediatric nephrology (Berlin, Germany) · 2015Article
- CFTR and TNR-CFTR expression and function in the kidney.Biophysical reviews · 2014Review
- Cystic fibrosis of pancreas and nephrotic syndrome: a rare association.Korean journal of pediatrics · 2013Article
- Newborn screening and renal disease: where we have been; where we are now; where we are going.Pediatric nephrology (Berlin, Germany) · 2012Review
- CFTR, mucins, and mucus obstruction in cystic fibrosis.Cold Spring Harbor perspectives in medicine · 2012Review
- Regulation of transport in the connecting tubule and cortical collecting duct.Comprehensive Physiology · 2012Review
- Article
- Decreased renal accumulation of aminoglycoside reflects defective receptor-mediated endocytosis in cystic fibrosis and Dent's disease.Pflugers Archiv : European journal of physiology · 2011Article
- Helical apolipoproteins of high-density lipoprotein enhance phagocytosis by stabilizing ATP-binding cassette transporter A7.Journal of lipid research · 2010Article
- Molecular and functional characterization of the cystic fibrosis transmembrane conductance regulator from the Australian common brushtail possum, Trichosurus vulpecula.Journal of comparative physiology. B, Biochemical, systemic, and environmental physiology · 2010Article
- CFTR structure and function: is there a role in the kidney?Biophysical reviews · 2009Review
- Alternate strategies of Hsp90 modulation for the treatment of cancer and other diseases.Current topics in medicinal chemistry · 2009Review
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Inactivation of the chloride channel cystic fibrosis transmembrane conductance regulator (CFTR) causes cystic fibrosis (CF). Although CFTR is expressed in the kidney, no overwhelming renal phenotype is associated with CF. Recent studies have shown that the level of CFTR mRNA in mouse kidney approaches that found in lung. CFTR is particularly abundant in the apical area of proximal tubule cells, where it co-distributes with the Cl(-)/H(+) exchanger ClC-5 and Rab5a in endosomes. The biological relevance of CFTR in proximal tubule endocytosis has been tested in CF mouse models and CF patients. Mice lacking CFTR show a defective receptor-mediated endocytosis, as evidenced by impaired uptake of (125)I-beta(2)-microglobulin, a decreased expression of the cubilin receptor in the kidney, and a significant excretion of cubilin and its low-molecular-weight ligands into the urine. Low-molecular-weight proteinuria (and particularly transferrinuria) is similarly detected in CF patients in comparison with normal controls or patients with chronic lung inflammation. These studies suggest that the functional loss of CFTR impairs the handling of low-molecular-weight proteins by the kidney, supporting a role of CFTR in receptor-mediated endocytosis in proximal tubule cells. The selective proteinuria should be integrated in the pathophysiology of multi-systemic complications increasingly observed in CF patients.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.