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The biochemistry, metabolism and inherited defects of the pentose phosphate pathway: A review
Author(s) -
Wamelink M. M. C.,
Struys E. A.,
Jakobs C.
Publication year - 2008
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-008-1015-6
Subject(s) - transaldolase , pentose phosphate pathway , transketolase , biochemistry , pentose , metabolic pathway , metabolism , human genetics , ribose , isomerase , glycolysis , triosephosphate isomerase , carbohydrate metabolism , biology , chemistry , enzyme , gene , fermentation
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.