The biochemistry, metabolism and inherited defects of the pentose phosphate pathway: A review
- First Online:
- Cite this article as:
- Wamelink, M.M.C., Struys, E.A. & Jakobs, C. J Inherit Metab Dis (2008) 31: 703. doi:10.1007/s10545-008-1015-6
- 2.1k Downloads
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.