[A flip-flop model of the chloride channel complex explains the dysregulation of the chloride flow in the plasmalemma of cells in cystic fibrosis].
The basic defect in cystic fibrosis is the chloride impermeability of the plasmalemma in different cells. A candidate for the chloride channel, thought to be affected in the syndrome, is "Porin 31HL" recently described by us. The molecule is i) expressed in the plasmalemma of different cells, it has ii) a molecular mass of 31,000 Daltons, it shows iii) high conductance in artificial membranes and it can be iv) modified by 4,4'-Diisothiocyanatostilbene-2,2'-disulfonate. A porin in the outer membrane of cells should furthermore v) be regulated by modulators. All these characters of "Porin 31HL" correspond to those given in literature for chloride channels. The regulation of the channels can be explained by a two component flip flop model.
Thinnes
F P
FP
Max-Planck-Institut für Experimentelle Medizin, Abteilung Immunchemie, Göttingen.
Babel
D
D
Hein
A
A
Jürgens
L
L
König
U
U
Schmid
A
A
Hilschmann
N
N
D017878:4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid; D012856:4-Acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic Acid / Q000031:analogs & derivatives / Q000494:pharmacology; D002463:Cell Membrane Permeability; D002712:Chlorides / Q000378:metabolism*; D003550:Cystic Fibrosis / Q000378:metabolism*; D006801:Humans; D007473:Ion Channels / Q000378:metabolism*; D008563:Membrane Lipids / Q000378:metabolism; D008565:Membrane Proteins / Q000302:isolation & purification / Q000378:metabolism; D008970:Molecular Weight; D018272:Porins; D050995:Voltage-Dependent Anion Channel 1; D050994:Voltage-Dependent Anion Channels
D002712:Chlorides; D007473:Ion Channels; D008563:Membrane Lipids; D008565:Membrane Proteins; D018272:Porins; C494762:VDAC1 protein, human; D050994:Voltage-Dependent Anion Channels; D012856:4-Acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic Acid; D050995:Voltage-Dependent Anion Channel 1; D017878:4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid
Ein Flip-Flop-Modell des Chlorid-Kanal-Komplexes erklärt die Fehlregulation des Chloridflusses am Plasmalemm von Zellen bei der cystischen Fibrose.