Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
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SHR Neuro Krebs Kardio Lipid Stoffw Microb
Plecko, B; Hoeger, H; Jakobs, C; Struys, E; Stromberger, C; Leschnik, M; Muehl, A; Stoeckler-Ipsiroglu, S.
Pipecolic acid concentrations in brain tissue of nutritionally pyridoxine-deficient rats.
J Inherit Metab Dis. 2005; 28(5):689-693
Doi: 10.1007/s10545-005-0071-4
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- Plecko Barbara
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- Abstract:
- 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Animal Nutritional Physiological Phenomena -
- Animals -
- Brain - metabolism
- Disease Models, Animal -
- Epilepsy - etiology
- Epilepsy - pathology
- Female -
- Male -
- Metabolism, Inborn Errors - diagnosis
- Metabolism, Inborn Errors - metabolism
- Pipecolic Acids - blood
- Pipecolic Acids - cerebrospinal fluid
- Pipecolic Acids - metabolism
- Pyridoxal Phosphate - metabolism
- Pyridoxine - deficiency
- Pyridoxine - metabolism
- Rats -
- Time Factors -
- Vitamin B 6 - metabolism