CFTR (Cystic fibrosis transmembrane conductance regulator, ATP-binding cassette sub-family C, member 7) is a membrane-associated, N-linked glycoprotein.
Immunogen
synthetic peptide corresponding to amino acids 103-117 of human CFTR protein. The sequence is 100% conserved in human, rabbit, and monkey. There is a one amino acid substitution in rat, bovine, and sheep.
Application
Anti-CFTR antibody produced in rabbit is suitable for immunocytochemistry at a concentration of 1μg/mL using HEK293 cells overexpressing human CFTR.
Biochem/physiol Actions
Cystic fibrosis transmembrane conductance regulator (CFTR) gene is associated with congenital bilateral absence of the vas deferens (CBAVD) and causes the genital form of cystic fibrosis (CF). The CFTR gene may also be involved in the etiology of male infertility. It may be useful for assisting reproduction technology. There is nearly 2000 cystic fibrosis transmembrane regulator (CFTR) mutations that are associated with cystic fibrosis (CF).
Physical form
Solution in phosphate buffered saline containing 1.0 mg/mL bovine serum albumin and 0.05 % sodium azide.
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The gene associated with cystic fibrosis (CF) encodes a membrane-associated, N-linked glycoprotein called CFTR. Mutations were introduced into CFTR at residues known to be altered in CF chromosomes and in residues believed to play a role in its function. Examination
The cystic fibrosis transmembrane conductance regulator (CFTR) functions to regulate both Cl- and Na+ conductive pathways; however, the cellular mechanisms whereby CFTR acts as a conductance regulator are unknown. CFTR and outwardly rectifying Cl- channels (ORCCs) are distinct channels but
Recent advances in the management of cystic fibrosis (CF) target underlying defects in the CF transmembrane conductance regulator (CFTR) protein, but efficacy analyses remain limited to specific genotype-based subgroups. Patient-derived model systems may therefore aid in expanding access to these
High incidence of mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene is associated with congenital bilateral absence of the vas deferens (CBAVD) and is considered as the genital form of cystic fibrosis (CF). The CFTR gene may also
There are nearly 2000 cystic fibrosis transmembrane regulator (CFTR) mutations that cause cystic fibrosis (CF). These mutations are classified into six classes; on the one hand, the first three classes cause severe disease involvement in early childhood, on the other
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