El-Agnaf O M, Guthrie D J, Walsh D M, Irvine G B
Peptide and Protein Engineering Centre, School of Biology & Biochemistry, The Queen's University of Belfast, UK.
Eur J Biochem. 1998 Sep 15;256(3):560-9. doi: 10.1046/j.1432-1327.1998.2560560.x.
Alzheimer's beta-amyloid peptide (Abeta) is a 39- to 43-amino-acid peptide that is the major component of neuritic plaques found in Alzheimer's disease (AD). The central region of Abeta plays a crucial role in many of its properties, including aggregation, neurotoxicity, proteolytic processing and interactions with other proteins, such as apolipoprotein E. Two mutations in this region, Ala21-->Gly and Glu22-->Gln, give rise to early onset forms of disease. We have studied several peptides based on the central region of Abeta in order to clarify the influence of specific amino acid residues on physicochemical behaviour. To avoid difficulties due to oxidation of Met35, the latter was replaced by the amino acid isostere, norleucine (Ahx), giving [Ahx35]Abeta-(25-35)-amide as a prototype structure. To this prototype, addition of pairs of amino acid residues from the sequence of Abeta, forming the corresponding 23-, 21- and 19-35 derivatives, resulted in peptides that aggregated to form fibrils of diameter 6-10 nm. The rate of aggregation was more rapid as peptide length increased. Circular dichroism spectra of aged solutions of peptides revealed that aggregation was accompanied by a transition from random structure to beta sheet for some, but not all, peptides. The mutation from Ala to Gly at position 21 increased the rate of aggregation and altered the tendency to adopt secondary structure in the direction away from alpha helix and towards beta sheet. In individuals with the Ala21-->Gly mutation, these results would suggest that truncated species with N-termini in the region containing residues 17-20 would be more amyloidogenic than the wild type homologues.
阿尔茨海默病β-淀粉样肽(Aβ)是一种由39至43个氨基酸组成的肽,是阿尔茨海默病(AD)中神经炎性斑块的主要成分。Aβ的中央区域在其许多特性中起着关键作用,包括聚集、神经毒性、蛋白水解加工以及与其他蛋白质(如载脂蛋白E)的相互作用。该区域的两个突变,Ala21→Gly和Glu22→Gln,会引发早发型疾病。我们研究了几种基于Aβ中央区域的肽,以阐明特定氨基酸残基对物理化学行为的影响。为避免因Met35氧化带来的困难,将其替换为氨基酸等排体正亮氨酸(Ahx),得到[Ahx35]Aβ-(25 - 35)-酰胺作为原型结构。在此原型基础上,从Aβ序列中添加成对的氨基酸残基,形成相应的23、21和19 - 35衍生物,得到的肽会聚集形成直径为6 - 10 nm的纤维。随着肽长度增加,聚集速率更快。肽老化溶液的圆二色光谱显示,对于一些(但并非全部)肽,聚集伴随着从无规结构向β折叠的转变。21位从Ala突变为Gly会增加聚集速率,并改变二级结构的形成倾向,使其远离α螺旋并趋向β折叠。对于携带Ala21→Gly突变的个体,这些结果表明,N端位于包含残基17 - 20区域的截短型物种比野生型同源物更具淀粉样变性。