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[PDF] Top 20 Calcium-activated chloride in cystic fibrosis

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Calcium-activated chloride in cystic fibrosis

Calcium-activated chloride in cystic fibrosis

... 1, in freshly isolated nasal cells from F508del-homozygous CF patients and non-CF healthy ...channels in cell lines stably expressing wild type (wt) or F508del-CFTR. In both cases no increase ... See full document

160

Presynaptic Localization and Possible Function of Calcium-Activated Chloride Channel Anoctamin 1 in the Mammalian Retina.

Presynaptic Localization and Possible Function of Calcium-Activated Chloride Channel Anoctamin 1 in the Mammalian Retina.

... ANO1 in retinal neurons. In the OPL, cone pedicles and rod spherules were labeled for ANO1, whereas invaginating ON-bipolar and horizontal cell dendrites, which are postsynaptic elements located at the ... See full document

11

Improvement of chloride transport defect by gonadotropin-releasing hormone (GnRH) in cystic fibrosis epithelial cells.

Improvement of chloride transport defect by gonadotropin-releasing hormone (GnRH) in cystic fibrosis epithelial cells.

... expression in cells and to study their modulation after GnRH ...eluted in RNase-free water and the concentration was determinated using a nano- photometer (Implen GmbH, Mu¨nchen, ...assessed in a two ... See full document

14

Vitamin K status in cystic fibrosis patients

Vitamin K status in cystic fibrosis patients

... disease in the Caucasian popu- lation [Hecker and Aris ...mutation in the CFTR gene (cystic fi- brosis transmembrane conductance ...a chloride ion channel found in epithelial tissues ... See full document

5

Adeno-associated virus for cystic fibrosis gene therapy

Adeno-associated virus for cystic fibrosis gene therapy

... evaluated in CF bronchial ...defective chloride transport in vitro in a CF bronchial cell line, as well as the hyper-inflammatory lung phenotype in Pseudomonas-agarose bead challenged ... See full document

9

Rev. Hosp. Clin.  vol.58 número5

Rev. Hosp. Clin. vol.58 número5

... patients. Cystic fibrosis is routinely diagnosed by clinical symptoms and sweat test- ing; genotyping was not routinely per- formed before 1998 except for selected or atypical ...sweat chloride ... See full document

3

The Cystic Fibrosis Transmembrane Regulator (CFTR) in the Kidney

The Cystic Fibrosis Transmembrane Regulator (CFTR) in the Kidney

... expressed in the renal medulla (Morales et ...expressed in Xenopus oocytes or mammalian cells, showed cAMP-dependent single chloride channel properties like those of the wild type CFTR, but with ... See full document

8

Abordagem nutricional na Fibrose Quística em idade pediátrica.

Abordagem nutricional na Fibrose Quística em idade pediátrica.

... Cystic Fibrosis (CF) is an autosomal recessive genetic pathology that mainly affects the Caucasian ...the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) ...implicated in the ... See full document

28

Genotype-phenotype correlation in cystic fibrosis patients bearing [H939R;H949L] allele

Genotype-phenotype correlation in cystic fibrosis patients bearing [H939R;H949L] allele

... sweat chloride tests (> 60 mEq/L) by Gibson and Cooke procedure, and the identification of two CF-disease causing mutations in trans (Rosenstein and Cutting, 1998; Kulczycki et ...volume in one ... See full document

5

Living in constant crisis--when stress management becomes the problem.

Living in constant crisis--when stress management becomes the problem.

... There were already reports of possible disadvantages of chronic stress response activation, but this paper clarifies the situation. The study concentrates initially on F508del-CFTR, a very common cystic ... See full document

2

Cystic fibrosis, are we missing in India?

Cystic fibrosis, are we missing in India?

... that Cystic fibrosis (CF), is now a pan ethnic ...survival in CF patients from developing countries are early age of onset of symptoms, severe malnutrition at the time of diagnosis and frequent ... See full document

5

Proteomic identification of calumenin as a G551D-CFTR associated protein.

Proteomic identification of calumenin as a G551D-CFTR associated protein.

... Cystic fibrosis (CF) is the most common lethal autosomal recessive disease in the Caucasian ...mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) ... See full document

10

Variation in MSRA modifies risk of neonatal intestinal obstruction in cystic fibrosis.

Variation in MSRA modifies risk of neonatal intestinal obstruction in cystic fibrosis.

... production in intestinal obstruction in CF mice at the time of weaning have been supported by the manipulation of expression of two ...genes. In one study, reduced expression of the sodium hydrogen ... See full document

10

Pseudomonas aeruginosa diversification during infection development in cystic fibrosis lungs

Pseudomonas aeruginosa diversification during infection development in cystic fibrosis lungs

... defects in genes involved in DNA-repair or error-avoidance system ...as in mutS or mutL genes that increase the rate of mutation up to a 1000 fold, being these the most frequent mutations ...change ... See full document

108

Diagnosis, Prognosis and Personalized Treatment of Cystic Fibrosis

Diagnosis, Prognosis and Personalized Treatment of Cystic Fibrosis

... From Portugal, to Miquéias for being supportive of my struggle, for all the reality checks, for sharing your experience with me and for saving me a position in your future lab (I will not forget this!). To ... See full document

90

Microbiota present in cystic fibrosis lungs as revealed by whole genome sequencing.

Microbiota present in cystic fibrosis lungs as revealed by whole genome sequencing.

... methods in parallel to detect as many microbial groups as possible, we cannot exclude biases in the proportions of microbiota members due to varying efficiencies of DNA ...biased in this respect ... See full document

7

Surfactant protein a in cystic fibrosis: supratrimeric structure and pulmonary outcome.

Surfactant protein a in cystic fibrosis: supratrimeric structure and pulmonary outcome.

... involved in surfactant metabolism and recognizes pathogen-associated molec- ular patterns on microorganisms, resulting in aggregation, opsonization or permeabilization of microorganisms and facilita- tion ... See full document

7

Plasma sCD14 as a biomarker to predict pulmonary exacerbations in cystic fibrosis.

Plasma sCD14 as a biomarker to predict pulmonary exacerbations in cystic fibrosis.

... increase in sCD14, CRP, MCP-1 and MPO levels when transitioning from a stable to exacerbation health state, some individual patients had no change or even a slight decrease in biomarker levels (Figure ... See full document

7

Clinical significance in non-cystic fibrosis bronchiectasis followed in a real practice

Clinical significance in non-cystic fibrosis bronchiectasis followed in a real practice

... non-cystic fibrosis bronchiectasis mainly post-infection or post-tuberculosis in origin are characterized by a low educational level, excessive cough, sputum, dyspnea, muscle fatigue, an obstructive ... See full document

10

Dysregulated proinflammatory and fibrogenic phenotype of fibroblasts in cystic fibrosis.

Dysregulated proinflammatory and fibrogenic phenotype of fibroblasts in cystic fibrosis.

... whether fibrosis is affected in CF lungs, we applied an in vivo mouse model of pulmonary fibrosis induced by bleomycin [12], a glycopeptide antibiotic commonly used as cancer ...high in ... See full document

10

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