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CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity
BackgroundThe primary underlying defect in cystic fibrosis (CF) is disrupted ion transport in epithelia throughout the body. It is unclear if...
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Acquired CFTR dysfunction and dense distribution of ionocytes in nasal mucosa of children with CRS
BackgroundIonocytes are rare cells in airway epithelium characterized by a high expression of CFTR.
ObjectivesTo investigate the morphology and...
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Androgen-induced upregulation of CFTR in pancreatic β-cell contributes to hyperinsulinemia in PCOS model
PurposePolycystic ovarian syndrome (PCOS) is an endocrine-metabolic condition affecting 5–10% of reproductive-aged women and characterized by...
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Expression of CFTR, a hallmark gene of ionocytes, is downregulated in salivary glands of Sjögren’s syndrome patients
IntroductionThe autoimmune exocrinopathy, Sjögren’s syndrome (SjS), is associated with secretory defects in salivary glands. The cystic fibrosis...
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Rhinorrhea and increased chloride secretion through the CFTR chloride channel-a systematic review
PurposeAllergic and non-allergic rhinorrhea in the forms of acute or chronic rhinosinusitis can mean a watery nasal discharge that is disabling....
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Cystic fibrosis transmembrane conductance regulator (CFTR): beyond cystic fibrosis
BackgroundThe cystic fibrosis transmembrane conductance regulator ( CFTR ) gene has been traditionally linked to cystic fibrosis (CF) inheritance in...
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Effects of CFTR-modulator triple therapy on sinunasal symptoms in children and adults with cystic fibrosis
PurposeSinunasal symptoms and chronic rhinusinutitis are common in patients with cystic fibrosis. Cystic fibrosis transmembrane regulator (CFTR)...
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Cystic fibrosis and CFTR-related disorder with electrolyte imbalance at diagnosis: clinical features and outcome in an Italian cohort
There is limited information available on the clinical data, sweat test trends, and outcomes of individuals with cystic fibrosis (CF) who present...
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The impact of CFTR modulator triple therapy on type 2 inflammatory response in patients with cystic fibrosis
BackgroundTreatment of cystic fibrosis (CF) has been revolutionized by the use of cystic fibrosis transmembrane conductance regulator (CFTR) protein...
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Targeted quantitation of CFTR protein expression in vivo using immunoprecipitation & parallel reaction monitoring tandem mass spectrometry
BackgroundThe cystic fibrosis transmembrane conductance regulator (CFTR) protein is a low-abundance membrane protein. The dysfunction of CFTR protein...
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A novel role for CFTR interaction with LH and FGF in azoospermia and epididymal maldevelopment caused by cryptorchidism
Cryptorchidism occurs frequently in children with cystic fibrosis. Among boys with cryptorchidism and abrogated mini-puberty, the development of the...
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CFTR Modulator Therapy and Its Impact on Lung Transplantation in Cystic Fibrosis
Cystic fibrosis (CF) is the most common autosomal recessive disorder in Caucasian people and is caused by mutations in the gene encoding for the CF...
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Mutational Spectrum of the CFTR Gene in the Kazakhstan Population
ObjectiveTo study the frequency and spectrum of CFTR gene variants in different ethnic groups of Kazakhstan.
MethodsWe reviewed the records of 58...
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Molecular analysis of CFTR gene mutations among Iraqi cystic fibrosis patients
BackgroundCystic fibrosis (CF) is an autosomal recessive multisystem disease that results from mutation(s) of the cystic fibrosis transmembrane...
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Cystic Fibrosis-Related Gut Dysbiosis: A Systematic Review
Background and AimsCystic Fibrosis (CF) is associated with gut dysbiosis, local and systemic inflammation, and impaired immune function. Gut...
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Truncating PICK1 Variant Identified in Azoospermia Affected Mitochondrial Dysfunction in Knockout Mice
ObjectiveThe protein interacting with C kinase 1 ( PICK1 ) plays a critical role in vesicle trafficking, and its deficiency in sperm cells results in...
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Rationale and design of the HIT-CF organoid study: stratifying cystic fibrosis patients based on intestinal organoid response to different CFTR-modulators
BackgroundCystic fibrosis is a rare recessive monogenic disease caused by loss-of-function mutations in the Cystic Fibrosis Transmembrane Conductance...
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Safety of chronic hypertonic bicarbonate inhalation in a cigarette smoke-induced airway irritation guinea pig model
BackgroundCystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD) are often associated with airway fluid acidification. Mutations in...
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Aetiology of Significant Liver Test Abnormalities in a Single-Centre Cohort of People with Cystic Fibrosis Exposed to Elexacaftor/Tezacaftor/Ivacaftor, Utilizing the Updated RUCAM
BackgroundThe cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulator elexacaftor/tezacaftor/ivacaftor (E/T/I) has been associated...
