Gene transfer for cystic fibrosis

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Gene transfer for cystic fibrosis.

years ago the cystic fibrosis (CF) gene was cloned. The discovery of the gene encoding the CF transmembrane conductance regulator (CFTR) Cl – channel marked the opening of Chapter 1 in the development of gene transfer as a treatment for this common autosomal recessive disease. One year later, Chapter 1 ended when expression of CFTR in CF epithelial cells corrected the CF Cl – transport defect. ...

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Gene Transfer to Cystic Fibrosis Airway Epithelia

Cationic lipids show promise as vectors for transfer of CFTR cDNA to airway epithelia of patients with cystic fibrosis (CF). However, previous studies have not compared the effect of DNA–lipid to DNA alone. Recently, we developed a formulation of plasmid encoding CFTR (pCF1CFTR) and cationic lipid (GL-67:DOPE) that generated greater gene transfer in mouse lung than previously described DNA–lipi...

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Barriers and recent advances in non-viral vectors targeting the lungs for cystic fibrosis gene therapy

Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in CFTR genes that affect chloride ion channel. The CF is a good nominee for gene therapy as the asymptomatic carriers are phenotypically normal, and the desired cells are accessible for vector delivery. Gene therapy shows promising effects involving the correction of gene or replacement of the mutant gene with the func...

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Prevalence of Cystic Fibrosis Trans-membrane Conductance Regulator Gene common mutations in children with cystic fibrosis in Isfahan, Iran

Background: Cystic fibrosis (CF) is the most common lethal genetic disorder of Cystic Fibrosis Trans-membrane Conductance (CFTR) Regulator gene mutations. We aimed to investigate common mutations in CF patients and to assess its possible relationship with clinical presentations. Materials and Methods: This cross sectional study was conducted on 36 CF patients who were referred to a tertiary ped...

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Gene therapy for cystic fibrosis.

Following the cloning of the cystic fibrosis (CF) gene, in vitro studies rapidly established the feasibility of gene therapy for this disease. Unlike ex vivo approaches that have been utilized for other genetic diseases such as adenosine deaminase deficiency, gene therapy for CF will likely require direct in vivo delivery of gene transfer vectors to the airways of patients with CF. Hence, major...

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ژورنال

عنوان ژورنال: Journal of Clinical Investigation

سال: 1999

ISSN: 0021-9738

DOI: 10.1172/jci8634