溶酶体寡糖降解
中文名称
通路描述
N-糖基化是真核生物蛋白质在ER腔中发生的最常见的共翻译和翻译后修饰之一。N-糖基化在蛋白质的折叠以及N-糖基化蛋白质的细胞内或细胞间运输中发挥关键作用。ER中的质量控制机制将错误折叠的蛋白质筛除,正确折叠的蛋白质则继续其正常功能。错误折叠的N-糖基化产物被导出至细胞质,降解过程由此开始。一旦未折叠的蛋白质从寡糖上被切割下来(形成游离寡糖,fOS),在细胞质中(Suzuki & Harada 2014)以及在溶酶体中(Aronson & Kuranda 1989, Winchester 2005)分步降解甘露糖残基,最终导致完全降解。降解必须彻底,以避免因残留小片段(如二聚体)而导致的溶酶体贮积病。
英文描述
Phosphate bond hydrolysis by NUDT proteins Enzymes that belong to the NUDT (Nudix) superfamily catalyze the hydrolysis of phosphodiester bonds in molecules including nucleoside triphosphates and diphosphates and nucleotide sugars. Family members are defined by the presence of an amino acid sequence motif shared with the E. coli MutT gene product, and are involved in diverse physiological processes (Mildvan et al. 2005; McLennan 2006).The hydrolysis of nucleoside di and triphosphates whose purine bases have been oxidized, deaminated, or methylated may protect the cell from the mutational damage that would occur if modified deoxyribonucleotides were incorporated into DNA and from the aberrant protein synthesis that would occur if modified ribonucleotides were incorporated into mRNA (Iyama et al. 2010; Takagi et al. 2012). The hydrolysis of ADP ribose may prevent the aberrant spontaneous ADP ribosylation of cellular proteins that could occur were this molecule to accumulate to high levels in the cell (Perraud et al. 2003; Shen et al. 2003).
所含基因
4 个基因