The biochemistry, metabolism and inherited defects of the pentose phosphate pathway: A review

The biochemistry, metabolism and inherited defects of the pentose phosphate pathway: A review
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DOI:
10.1007/s10545-008-1015-6
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发表时间:
2008-12-01
影响因子:
4.2
通讯作者:
Jakobs, C.
Jakobs, C.
中科院分区:
医学2区
文献类型:
--
作者:
Wamelink, M. M. C.;Struys, E. A.;Jakobs, C.

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最近在戊糖磷酸途径(PPP)的可逆部分发现了两个缺陷(核糖-5-磷酸异构酶缺陷和转醛缩酶缺陷),这激发了人们对该途径的兴趣。在这篇综述中,我们描述了PPP的功能,它与其他碳水化合物代谢途径的关系,并概述了PPP可逆部分的代谢缺陷。
The recent discovery of two defects (ribose-5-phosphate isomerase deficiency and transaldolase deficiency) in the reversible part of the pentose phosphate pathway (PPP) has stimulated interest in this pathway. In this review we describe the functions of the PPP, its relation to other pathways of carbohydrate metabolism and an overview of the metabolic defects in the reversible part of the PPP.