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Lysosomal oligosaccharide catabolism

Reactome ID: R-HSA-8853383

中文名称

FGFR2 融合介导的信号通路

通路描述

FGFR2 融合已在肺癌、乳腺癌、甲状腺癌和胆管癌等癌症中被鉴定出来(Wu et al, 2013; Seo et al, 2012; Arai et al, 2013)。在所有 FGF 受体中,FGFR2 显示出最广泛的 3'融合伙伴范围,包括 BICC1、AHCYL1、CIT、CCDC6、CASP7、AFF3、OFD1 和 CCAR2。许多这些融合伙伴含有二聚化结构域,表明这些融合可能表现出构象依赖的配体无关激活(Wu et al, 2013; Arai et al, 2013; Seo et al, 2012; 综述 Parker et al, 2014)。
英文描述
Lysosomal oligosaccharide catabolism N-Glycosylation is one of the most common co- and posttranslational modifications of eukaryotic proteins occurring in the ER lumen. N-glycosylation plays pivotal roles in protein folding and intra- or inter-cellular trafficking of N-glycosylated proteins. Quality control mechanisms in the ER sift out incorrectly-folded proteins from correctly-folded proteins, the former then destined for degradation. Incorrectly-folded N-glycans are exported to the cytosol where the process of degradation begins. Once the unfolded protein is cleaved from the oligosaccharide (forming free oligosaccharides, fOS), step-wise degradation of mannose moieties, both in the cytosol (Suzuki & Harada 2014) and then in the lysosome (Aronson & Kuranda 1989, Winchester 2005), results in complete degradation. Breakdown must be complete to avoid lysosomal storage diseases that occur when fragments as small as dimers are left undigested.

所含基因

4 个基因