Benharouga M, Fritsch J, Goossens M, Edelman A
INSERM U, 323, CHU Necker, Paris, France.
C R Acad Sci III. 1996 Nov;319(11):1019-25.
The cystic fibrosis transmembrane conductance regulator (CFTR) is a transmembrane protein that is expressed in several epithelia, including kidney tubules. Mutations in CFTR (a PKA-chloride channel and/or regulator of other epithelial channels) give rise to the clinical manifestation of cystic fibrosis, and result in the synthesis of mutated proteins responsible for altering ion transport across secretory epithelia. The low abundance of endogenous CFTR makes a difficult to purify enough of the native protein to prepare anti-CFTR antibodies. We have used differential centrifugation to prepare cortical brush border membrane vesicles from pig kidney, cBBMV, and developed a method for the partial purification of CFTR. This is the first step in the isolation of native CFTR. The results show that CFTR is present in cBBMV. The purified protein will provide a clearer picture of the biophysical and biochemical properties of native CFTR.
囊性纤维化跨膜传导调节因子（CFTR）是一种跨膜蛋白，在包括肾小管在内的多种上皮组织中表达。CFTR（一种蛋白激酶A - 氯离子通道和/或其他上皮通道的调节因子）的突变会引发囊性纤维化的临床表现，并导致负责改变离子跨分泌上皮运输的突变蛋白的合成。内源性CFTR的丰度较低，难以纯化足够的天然蛋白来制备抗CFTR抗体。我们利用差速离心法从猪肾制备了皮质刷状缘膜囊泡（cBBMV），并开发了一种部分纯化CFTR的方法。这是分离天然CFTR的第一步。结果表明CFTR存在于cBBMV中。纯化后的蛋白将更清晰地展现天然CFTR的生物物理和生化特性。
