Journal of Inherited Metabolic Disease

, Volume 31, Issue 6, pp 703–717

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

Metabolic Dissertation

DOI: 10.1007/s10545-008-1015-6

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


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.

Copyright information

© Springer Science+Business Media B.V. 2008

Authors and Affiliations

  1. 1.Metabolic Unit, Department of Clinical ChemistryVU University Medical CenterAmsterdamThe Netherlands

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