Thinnes F P, Babel D, Hein A, Jürgens L, König U, Schmid A, Hilschmann N
Max-Planck-Institut für Experimentelle Medizin, Abteilung Immunchemie, Göttingen.
Klin Wochenschr. 1991 May 3;69(7):283-8. doi: 10.1007/BF01644755.
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.
囊性纤维化的基本缺陷是不同细胞中质膜对氯离子的不通透性。我们最近描述的“孔蛋白31HL”被认为是该综合征中受影响的氯离子通道候选者。该分子具有以下特性:i)在不同细胞的质膜中表达;ii)分子量为31,000道尔顿;iii)在人工膜中具有高电导率;iv)可被4,4'-二异硫氰基芪-2,2'-二磺酸盐修饰。此外,细胞外膜中的孔蛋白v)应受调节剂调控。“孔蛋白31HL”的所有这些特性与文献中给出的氯离子通道特性相符。通道的调控可用双组分翻转模型来解释。