Regulation of dolichol-linked glycosylation
Regulation of dolichol-linked glycosylation
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多醇连接糖基化的调节
作者：
M. Welti
在大多数先天性糖基化疾病中，多糖前体GlcNAc2Man9Glc3在聚戊二醇载体多酰基焦磷酸上的组装受到损害。由于n链糖基化对生命至关重要，大多数先天性糖基化疾病都代表酶活性的部分丧失。因此，沿糖基化途径增加底物的可用性有利于增加受损酶的产物形成。最近，我们发现可以通过抑制角鲨烯的生物合成来增加醇的可用性和改善n -链糖基化。本文综述了目前在生物合成多醇链聚糖方面的知识，以及n链糖基化的缺陷。此外，研究了针对醇和醇联聚糖生物合成的治疗方法。
In the majority of congenital disorders of glycosylation, the assembly of the glycan precursor GlcNAc2Man9Glc3 on the polyprenol carrier dolichyl-pyrophosphate is compromised. Because N-linked glycosylation is essential to life, most types of congenital disorders of glycosylation represent partial losses of enzymatic activity. Consequently, increased availability of substrates along the glycosylation pathway can be beneficial to increase product formation by the compromised enzymes. Recently, we showed that increased dolichol availability and improved N-linked glycosylation can be achieved by inhibition of squalene biosynthesis. This review summarizes the current knowledge on the biosynthesis of dolichol-linked glycans with respect to deficiencies in N-linked glycosylation. Additionally, perspectives on therapeutic treatments targeting dolichol and dolichol-linked glycan biosynthesis are examined.
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