459810-37-8Relevant articles and documents
Synthesis of a molecular mimic of the Glc1Man9 oligoside as potential inhibitor of calnexin binding to ΔF508 CFTR protein
Cherif, Slim,Leach, Michael R.,Williams, David B.,Monneret, Claude
, p. 1237 - 1240 (2002)
Deletion of phenylalanine at position 508 of the CFTR protein is associated with a severe form of cystic fibrosis. Biosynthetic arrest of the misfolded ΔF508 CFTR protein in the endoplasmic reticulum is due to prolonged interaction with protein chaperones. In order to overcome this retention and thereby restore the delivery of the protein to the plasma membrane, a molecular mimic of the glycoprotein oligoside moiety has been designed and synthesized. Ability of this mimic to inhibit the binding of the natural Glc1Man9GlcNAc oligoside to calnexin has been measured.