Multidrug resistance protein-6 (MRP6) in human dermal fibroblasts. Comparison between cells from normal subjects and from Pseudoxanthoma elasticum patients

• Published in: Matrix biology Vol. 22; no. 6; pp. 491 - 500
• Main Authors: Boraldi, F., Quaglino, D., Croce, M.A., Garcia Fernandez, M.I., Tiozzo, R., Gheduzzi, D., Bacchelli, B., Pasquali Ronchetti, I.
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.11.2003
• Subjects: ABCC6; Adult; Benzbromarone - pharmacology; Cell Count; Cell Division; Chlorambucil - pharmacology; Female; Fibroblast; Fibroblasts - cytology; Fibroblasts - drug effects; Fibroblasts - metabolism; Fluoresceins - pharmacokinetics; Fluoresceins - pharmacology; Fluorescent Dyes - pharmacokinetics; Fluorescent Dyes - pharmacology; Humans; Indomethacin - pharmacology; Lysosomes - chemistry; Male; Membrane Transport Proteins - physiology; Membrane transporter; Microscopy, Fluorescence; Middle Aged; MRP6; Multidrug Resistance-Associated Proteins - antagonists & inhibitors; Multidrug Resistance-Associated Proteins - physiology; Propionates - pharmacology; Pseudoxanthoma elasticum; Pseudoxanthoma Elasticum - metabolism; Pseudoxanthoma Elasticum - physiopathology; Quinolines - pharmacology; Skin - metabolism; Skin - physiopathology; Verapamil - pharmacology; Vinblastine - pharmacology; CHB, chlorambucil; NEM-GS, N-ethyl-maleimide- S-glutathione; PXE, Pseudoxanthoma elasticum (on-line Mendelian inheritance in man 264800, MIM 177850); DMEM, Dulbecco modified eagle medium; ABCC6; BNZ, benzbromarone; FBS, fetal bovine serum; PBS, phosphate buffered saline; MK571, 3-(3-(2-(7-choro-2 quinolinyl) ethenyl)phenyl ((3-dimethyl amino-3-oxo-propyl)thio) methyl) propanoic acid; MRP6; VPL, verapamil; Calcein-AM, calcein acetoxy-methyl-ester; IDM, indomethacin; VBL, vinblastine; ABCC, ATP-binding cassette C; Pseudoxanthoma elasticum; MRP, multidrug resistance protein; Fibroblast; Membrane transporter; DMSO, dimethyl-sulphoxide

Multidrug resistance protein-6 (MRP6) is a membrane transporter whose deficiency leads to the connective tissue disorder Pseudoxanthoma elasticum (PXE). In vitro dermal fibroblasts from normal and PXE subjects, homozygous for the R1141X mutation, were compared for their ability to accumulate and to...