Metabolic Dissertation

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

  • M. M. C. WamelinkAffiliated withMetabolic Unit, Department of Clinical Chemistry, VU University Medical Center Email author 
  • , E. A. StruysAffiliated withMetabolic Unit, Department of Clinical Chemistry, VU University Medical Center
  • , C. JakobsAffiliated withMetabolic Unit, Department of Clinical Chemistry, VU University Medical Center

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Summary

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.