Olson Linda J, Orsi Ramiro, Peterson Francis C, Parodi Armando J, Kim Jung-Ja P, D'Alessio Cecilia, Dahms Nancy M
†Department of Biochemistry, Medical College of Wisconsin, Milwaukee, Wisconsin 53226, United States.
‡Laboratory of Glycobiology, Fundación Instituto Leloir and Instituto de Investigaciones Bioquímicas de Buenos Aires-CONICET, Buenos Aires, Argentina.
Biochemistry. 2015 Jul 7;54(26):4097-111. doi: 10.1021/acs.biochem.5b00256. Epub 2015 Jun 24.
N-Glycans are modified as part of a quality control mechanism during glycoprotein folding in the endoplasmic reticulum (ER). Glucosidase II (GII) plays a critical role by generating monoglucosylated glycans that are recognized by lectin chaperones, calnexin and calreticulin. To understand how the hydrolytic activity of GIIα is enhanced by the mannose 6-phosphate receptor (MPR) homology domain (MRH domain) of its β subunit, we now report a 1.6 Å resolution crystal structure of the MRH domain of GIIβ bound to mannose. A comparison of ligand-bound and unbound structures reveals no major difference in their overall fold, but rather a repositioning of side chains throughout the binding pocket, including Y372. Mutation of Y372 inhibits GII activity, demonstrating an important role for Y372 in regulating GII activity. Comparison of the MRH domains of GIIβ, MPRs, and the ER lectin OS-9 identified conserved residues that are critical for the structural integrity and architecture of the carbohydrate binding pocket. As shown by nuclear magnetic resonance spectroscopy, mutations of the primary binding pocket residues and adjacent W409, all of which inhibit the activity of GII both in vitro and in vivo, do not cause a significant change in the overall fold of the GIIβ MRH domain but impact locally the stability of the binding pocket. W409 does not directly contact mannose; rather, its indole ring is stabilized by binding into a hydrophobic pocket of an adjacent crystallographic neighbor. This suggests that W409 interacts with a hydrophobic region of the GIIβ or GIIα subunit to modulate its effect on GII activity.
N-聚糖在内质网(ER)中糖蛋白折叠过程中作为质量控制机制的一部分被修饰。葡萄糖苷酶II(GII)通过生成被凝集素伴侣钙网蛋白和钙连蛋白识别的单葡萄糖基化聚糖发挥关键作用。为了解其β亚基的甘露糖6-磷酸受体(MPR)同源结构域(MRH结构域)如何增强GIIα的水解活性,我们现在报道了与甘露糖结合的GIIβ的MRH结构域的1.6 Å分辨率晶体结构。配体结合和未结合结构的比较显示它们的整体折叠没有重大差异,而是整个结合口袋中侧链的重新定位,包括Y372。Y372的突变抑制了GII活性,表明Y372在调节GII活性中起重要作用。GIIβ、MPR和内质网凝集素OS-9的MRH结构域的比较确定了对碳水化合物结合口袋的结构完整性和结构至关重要的保守残基。如核磁共振光谱所示,主要结合口袋残基和相邻的W409的突变,在体外和体内均抑制GII活性,但不会导致GIIβ MRH结构域的整体折叠发生显著变化,而是局部影响结合口袋的稳定性。W409不直接与甘露糖接触;相反,其吲哚环通过结合到相邻晶体学邻居的疏水口袋中而稳定。这表明W409与GIIβ或GIIα亚基的疏水区域相互作用以调节其对GII活性的影响。