Cystic fibrosis (CF) is caused by the functional expression defect of the CF transmembrane conductance regulator (CFTR) chloride channel at the apical plasma membrane. Impaired bacterial clearance and hyperactive innate immune response are hallmarks of the CF lung disease, yet the existence of and mechanism accounting for the innate immune defect that occurs before infection remain controversia...

Most CF (cystic fibrosis) results from deletion of a phenylalanine (F508) in the CFTR {CF transmembrane-conductance regulator; ABCC7 [ABC (ATP-binding cassette) sub-family C member 7]} which causes ER (endoplasmic reticulum) degradation of the mutant. Using stably CFTR-expressing BHK (baby-hamster kidney) cell lines we demonstrated that wild-type CTFR and the F508delCFTR mutant are cleaved into...

Suppose that $A$ is a semi-simple and commutative Banach algebra. In this paper we try to characterize the character space of the Banach algebra $C_{rm{BSE}}(Delta(A))$ consisting of all  BSE-functions on $Delta(A)$ where $Delta(A)$ denotes the character space of $A$. Indeed, in the case that $A=C_0(X)$ where $X$ is a non-empty locally compact Hausdroff space, we give a complete characterizatio...

The aim of this paper is to introduce and study a new concept ofstrong double $(A)_ {Delta}$-convergent sequence offuzzy numbers with respect to an Orlicz function and also someproperties of the resulting sequence spaces of fuzzy   numbers areexamined. In addition, we define the double$(A,Delta)$-statistical convergence of fuzzy  numbers andestablish some connections between the spaces of stron...

The present study was undertaken to evaluate the frequency and nature of mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene in infertile patients undergoing intracytoplasmic sperm injection. A total of 90 patients were screened for a panel of 10 mutations in the CFTR gene frequently involved in congenital absence of the vas deferens (CAVD); the patients included 14...

Activation of an apical Ca(2+)-activated Cl(-) channel (CaCC) triggers the secretion of saliva. It was previously demonstrated that CaCC-mediated Cl(-) current and Cl(-) efflux are absent in the acinar cells of systemic Tmem16A (Tmem16A Cl(-) channel) null mice, but salivation was not assessed in fully developed glands because Tmem16A null mice die within a few days after birth. To test the rol...

Cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel that belongs to the ATP binding cassette protein superfamily. Deletion of phenylalanine at position 508 (ΔF508) is the most common CF-associated mutation and is present in nearly 90% of CF patients. Currently, atomistic level studies are insufficient for understanding the mechanism by which the deletion of a single a...