Haddick Patrick C G, Larson Jessica L, Rathore Nisha, Bhangale Tushar R, Phung Qui T, Srinivasan Karpagam, Hansen David V, Lill Jennie R, Pericak-Vance Margaret A, Haines Jonathan, Farrer Lindsay A, Kauwe John S, Schellenberg Gerard D, Cruchaga Carlos, Goate Alison M, Behrens Timothy W, Watts Ryan J, Graham Robert R, Kaminker Joshua S, van der Brug Marcel
Department of Diagnostic Discovery, Genentech Inc., South San Francisco, CA, USA.
Department of Bioinformatics and Computational Biology, Genentech Inc., South San Francisco, CA, USA.
J Alzheimers Dis. 2017;56(3):1037-1054. doi: 10.3233/JAD-160524.
The common p.D358A variant (rs2228145) in IL-6R is associated with risk for multiple diseases and with increased levels of soluble IL-6R in the periphery and central nervous system (CNS). Here, we show that the p.D358A allele leads to increased proteolysis of membrane bound IL-6R and demonstrate that IL-6R peptides with A358 are more susceptible to cleavage by ADAM10 and ADAM17. IL-6 responsive genes were identified in primary astrocytes and microglia and an IL-6 gene signature was increased in the CNS of late onset Alzheimer's disease subjects in an IL6R allele dependent manner. We conducted a screen to identify variants associated with the age of onset of Alzheimer's disease in APOE ɛ4 carriers. Across five datasets, p.D358A had a meta P = 3 ×10-4 and an odds ratio = 1.3, 95% confidence interval 1.12 -1.48. Our study suggests that a common coding region variant of the IL-6 receptor results in neuroinflammatory changes that may influence the age of onset of Alzheimer's disease in APOE ɛ4 carriers.
白细胞介素-6受体(IL-6R)中常见的p.D358A变异体(rs2228145)与多种疾病风险相关,且与外周和中枢神经系统(CNS)中可溶性IL-6R水平升高有关。在此,我们表明p.D358A等位基因会导致膜结合型IL-6R的蛋白水解增加,并证明含有A358的IL-6R肽更易被ADAM10和ADAM17裂解。在原代星形胶质细胞和小胶质细胞中鉴定出了IL-6反应性基因,并且在晚发性阿尔茨海默病患者的中枢神经系统中,IL-6基因特征以IL6R等位基因依赖的方式增加。我们进行了一项筛查,以鉴定与APOEε4携带者中阿尔茨海默病发病年龄相关的变异体。在五个数据集中,p.D358A的meta P = 3×10-4,优势比 = 1.3,95%置信区间为1.12 - 1.48。我们的研究表明,IL-6受体的一个常见编码区变异体会导致神经炎症变化,这可能会影响APOEε4携带者中阿尔茨海默病的发病年龄。