(Q37288563)

19 August 2017

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title

CFTR and defective endocytosis: new insights in the renal phenotype of cystic fibrosis (English)

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19 August 2017

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2

Olivier Devuyst

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author name string

François Jouret

series ordinal

1

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19 August 2017

7 October 2008

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19 August 2017

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19 August 2017

457

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19 August 2017

1227-1236

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19 August 2017

6

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https://scigraph.springernature.com/pub.10.1007/s00424-008-0594-2

19 August 2017

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CF gene and cystic fibrosis transmembrane conductance regulator expression in autosomal dominant polycystic kidney disease

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Both the wild type and a functional isoform of CFTR are expressed in kidney.

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Processing and function of CFTR-DeltaF508 are species-dependent

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Renal tubule albumin transport

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Fertility issues in cystic fibrosis

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Endosomal hyperacidification in cystic fibrosis is due to defective nitric oxide-cylic GMP signalling cascade

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Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

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CFTR is required for PKA-regulated ATP sensitivity of Kir1.1 potassium channels in mouse kidney

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12 December 2020

Regulation of cystic fibrosis transmembrane regulator trafficking and protein expression by a Rho family small GTPase TC10

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12 December 2020

cAMP stimulates CFTR-like Cl- channels and inhibits amiloride-sensitive Na+ channels in mouse CCD cells

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Ion transport and cystogenesis: the paradigm of autosomal dominant polycystic kidney disease

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Subclinical anaemia of chronic disease in adult patients with cystic fibrosis.

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12 December 2020

CFTR mRNA and its truncated splice variant (TRN-CFTR) are differentially expressed during collecting duct ontogeny.

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12 December 2020

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

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12 December 2020

Genetics of hypercalciuric stone forming diseases

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Pseudo-Bartter's syndrome in cystic fibrosis

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Defective acidification of intracellular organelles in cystic fibrosis

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12 December 2020

Voltage-dependent electrogenic chloride/proton exchange by endosomal CLC proteins

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12 December 2020

Mice lacking renal chloride channel, CLC-5, are a model for Dent's disease, a nephrolithiasis disorder associated with defective receptor-mediated endocytosis.

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12 December 2020

Megalin Deficiency Offers Protection from Renal Aminoglycoside Accumulation

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Mutant (delta F508) cystic fibrosis transmembrane conductance regulator Cl- channel is functional when retained in endoplasmic reticulum of mammalian cells.

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12 December 2020

Chloride channel diseases resulting from impaired transepithelial transport or vesicular function

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12 December 2020

Loss of chloride channel ClC-5 impairs endocytosis by defective trafficking of megalin and cubilin in kidney proximal tubules

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12 December 2020

Regulation of plasma membrane recycling by CFTR.

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12 December 2020

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Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer

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Hyperacidification of cellubrevin endocytic compartments and defective endosomal recycling in cystic fibrosis respiratory epithelial cells

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ClC-5: role in endocytosis in the proximal tubule.

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Chloride and the endosomal-lysosomal pathway: emerging roles of CLC chloride transporters.

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Calcium-pump inhibitors induce functional surface expression of Delta F508-CFTR protein in cystic fibrosis epithelial cells

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Microscopic nephrocalcinosis in cystic fibrosis

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12 December 2020

Intra-renal and subcellular distribution of the human chloride channel, CLC-5, reveals a pathophysiological basis for Dent's disease

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12 December 2020

A role for CFTR in human autosomal dominant polycystic kidney disease

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12 December 2020

Variant cystic fibrosis phenotypes in the absence of CFTR mutations.

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12 December 2020

Functional iron deficiency in adults with cystic fibrosis

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12 December 2020

Structure and function of the CFTR chloride channel

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12 December 2020

ClC-5, the chloride channel mutated in Dent's disease, colocalizes with the proton pump in endocytotically active kidney cells

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12 December 2020

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12 December 2020

Chloride deficiency as a presentation or complication of cystic fibrosis

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12 December 2020

Identification of the cystic fibrosis gene: genetic analysis

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12 December 2020

Cadmium inhibits vacuolar H(+)-ATPase and endocytosis in rat kidney cortex.

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12 December 2020

The phenotypic consequences of CFTR mutations

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12 December 2020

Intracellular chloride channels: determinants of function in the endosomal pathway.

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12 December 2020

Cystic fibrosis is associated with a defect in apical receptor-mediated endocytosis in mouse and human kidney

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12 December 2020

Degradation of CFTR by the ubiquitin-proteasome pathway

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12 December 2020

Impaired acidification in early endosomes of ClC-5 deficient proximal tubule

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12 December 2020

Effects of dextran on lysosomal ultrastructure and protein digestion in renal proximal tubule

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12 December 2020

The functional cobalamin (vitamin B12)-intrinsic factor receptor is a novel complex of cubilin and amnionless

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12 December 2020

DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis

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12 December 2020

The purified ATPase from chromaffin granule membranes is an anion-dependent proton pump

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12 December 2020

New insights into cystic fibrosis: molecular switches that regulate CFTR

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12 December 2020

Comparative genomic sequence analysis of the human and mouse cystic fibrosis transmembrane conductance regulator genes

1 reference

12 December 2020

The amnionless gene, essential for mouse gastrulation, encodes a visceral-endoderm-specific protein with an extracellular cysteine-rich domain

1 reference

12 December 2020

Endocytosis provides a major alternative pathway for lysosomal biogenesis in kidney proximal tubular cells

1 reference

12 December 2020

ClC-5 Cl- -channel disruption impairs endocytosis in a mouse model for Dent's disease.

1 reference

12 December 2020

The Cl-/H+ antiporter ClC-7 is the primary chloride permeation pathway in lysosomes.

1 reference

12 December 2020

A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo

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12 December 2020

Regulated portals of entry into the cell

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12 December 2020

Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype

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12 December 2020

The association of nephrolithiasis with cystic fibrosis

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12 December 2020

The cystic fibrosis transmembrane regulator forms macromolecular complexes with PDZ domain scaffold proteins

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12 December 2020

A mouse model for the cystic fibrosis delta F508 mutation.

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12 December 2020

Early embryonic renal tubules of wild-type and polycystic kidney disease kidneys respond to cAMP stimulation with cystic fibrosis transmembrane conductance regulator/Na(+),K(+),2Cl(-) Co-transporter-dependent cystic dilation

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12 December 2020

Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

1 reference

12 December 2020

X-ray structure of a ClC chloride channel at 3.0 A reveals the molecular basis of anion selectivity

1 reference

12 December 2020

10.1007/S00424-008-0594-2

Identifiers

DOI

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19 August 2017

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PubMed ID

18839205