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Pipecolic acid concentrations in brain tissue of nutritionally pyridoxine‐deficient rats
Author(s) -
Plecko B.,
Hoeger H.,
Jakobs C.,
Struys E.,
Stromberger C.,
Leschnik M.,
Muehl A.,
StoecklerIpsiroglu S.
Publication year - 2005
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/s10545-005-0071-4
Subject(s) - pipecolic acid , pyridoxine , endocrinology , medicine , pyridoxal , chemistry , metabolism , epilepsy , biochemistry , amino acid , biology , enzyme , neuroscience
Summary Elevated concentrations of pipecolic acid have been reported in plasma and CSF of patients with pyridoxine‐dependent epilepsy, but its molecular background is unclear. To investigate any further association of pyridoxine and pipecolic acid metabolism, we have performed an animal trial and have measured the concentration of pipecolic acid in brain tissue of rats with nutritional pyridoxine deficiency and in control littermates. Concentrations of pyridoxal phosphate were significantly reduced in brain tissue of pyridoxine‐deficient rats ( p < 0.001), while concentrations of pipecolic acid were not significantly different from the normally nourished control group ( p = 0.3). These data indicate that a direct association of pyridoxine and pipecolic acid metabolism is unlikely. We therefore assume that the characteristic elevation of pipecolic acid in pyridoxine‐dependent epilepsy could rather be a secondary phenomenon with the primary defect of pyridoxine‐dependent epilepsy being located outside the pipecolic acid pathway.