Rabzelj Sabina, Viero Gabriella, Gutiérrez-Aguirre Ion, Turk Vito, Dalla Serra Mauro, Anderluh Gregor, Zerovnik Eva
Department of Biochemistry and Molecular Biology, JoZef Stefan Institute, Ljubljana, Slovenia.
FEBS J. 2008 May;275(10):2455-66. doi: 10.1111/j.1742-4658.2008.06390.x. Epub 2008 Apr 4.
Human stefin B, from the family of cystatins, is used as a model amyloidogenic protein in studies of the mechanism of amyloid fibril formation and related cytotoxicity. Interaction of the protein's prefibrillar oligomers/aggregates with predominantly acidic phospholipid membranes is known to correlate with cellular toxicity. In the present study, we measured membrane interaction of the prefibrillar and native states for three variants: the Y31 isoform studied previously, the wild-type protein and the G4R mutant; the latter is observed in progressive myoclonus epilepsy of type 1. In addition to using critical pressure and surface plasmon resonance, we assessed membrane permeabilization by calcein release and electrophysiological measurements. It was demonstrated for the first time that wild-type stefin B and the Y31 isoform are able to form pores in planar lipid bilayers, whereas G4R destroys the bilayer by a non pore-forming process. Similarities to other amyloidogenic proteins and the possible physiological implications of our findings are discussed.
人源抑半胱氨酸蛋白酶B属于胱抑素家族,在淀粉样原纤维形成机制及相关细胞毒性的研究中用作模型淀粉样蛋白。已知该蛋白的前原纤维寡聚体/聚集体与主要为酸性的磷脂膜之间的相互作用与细胞毒性相关。在本研究中,我们测量了三种变体的前原纤维状态和天然状态与膜的相互作用,这三种变体分别是:先前研究过的Y31异构体、野生型蛋白和G4R突变体;后者在1型进行性肌阵挛癫痫中出现。除了使用临界压力和表面等离子体共振外,我们还通过钙黄绿素释放和电生理测量评估了膜通透性。首次证明野生型抑半胱氨酸蛋白酶B和Y31异构体能够在平面脂质双分子层中形成孔,而G4R通过非成孔过程破坏双分子层。我们讨论了与其他淀粉样蛋白的相似性以及我们研究结果可能的生理意义。