TY - JOUR
TI - CFTR localization in native airway cells and cell lines expressing
wild-type or F508del-CFTR by a panel of different antibodies
AU - Carvalho-Oliveira, I
AU - Efthymiadou, A
AU - Malho, R
AU - Nogueira, P and
AU - Tzetis, M
AU - Kanavakis, E
AU - Amaral, MD
AU - Penque, D
JO - Journal of Histochemistry and Cytochemistry
PY - 2004
VL - 52
TODO - 2
SP - 193-203
PB - SAGE Publications Ltd
SN - 0022-1554, 1551-5044
TODO - 10.1177/002215540405200207
TODO - cystic fibrosis; CFTR; CFTR antibody; immunocytochemistry; nasal
brushing; epithelial cells
TODO - The intracellular localization of cystic fibrosis transmembrane
conductance regulator (CFTR) in native tissues is a major issue in the
study of mutation, processing, and trafficking effects in CFTR and in
the evaluation of therapeutic strategies in cystic fibrosis (CF). This
work evaluated the applicability of ten different antibodies (Abs) under
various fixation techniques for CFTR localization in fresh-brushed nasal
epithelial cells collected from CF patients homozygous for F508del and
control individuals. in parallel, the same Ab panel was also tested on
BHK cell lines overexpressing wild-type or F508del CFTR. The Abs
MATG1061, 169, Lis1, MP-CT1, CC24-R, MAB25031, and MAB1660 gave the best
detection of CFTR in the apical region (AR) of nasal tall columnar
epithelial (TCE) cells. The labeling pattern of these Abs was consistent
with the postulated processing defect of F508del CFTR because only a
minority of CF TCE cells present CFTR in the AR. In contrast, M3A7,
MM13-4, and L12B4 weakly react with the AR and stain almost exclusively
a cis-Golgi-like structure in the majority of CF and non-CF airway
cells. In BHK cells, all the Abs enabled distinction between wild-type
CFTR localization in cell membrane from F508del CFTR, which in these
cells is exclusively located in the endoplasmic reticulum.
ER -