Skip to main content
SpringerLink
Log in

Pipecolic acid concentrations in brain tissue of nutritionally pyridoxine-deficient rats

  • Published:
Journal of Inherited Metabolic Disease

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Battaglioli G, Rosen DR, Gospe SM, Martin DL (2000) Glutamate decarboxylase is not genetically linked to pyridoxine-dependent seizures. Neurology 55: 309–311.

    PubMed  CAS  Google Scholar 

  • Chang YF (1982) Lysine metabolism in the human and the monkey: demonstration of pipecolic acid formation in the brain and other organs. Neurochem Res 7: 577–588.

    Article  PubMed  CAS  Google Scholar 

  • Dancis J, Hutzler J (1982) Comparative rates of metabolism of pipecolic acid in several animal species. Comp Biochem Physiol 73B: 1011–1012.

    CAS  Google Scholar 

  • Guilarte T (1989) Regional changes in the concentrations of glutamate, glycine, taurine and GABA in the vitamin B-6 deficient developing rat brain: association with neonatal seizures. Neurochem Res 14: 889–897.

    PubMed  CAS  Google Scholar 

  • Kimura M, Kanehira K, Yokoi K (1996) Highly sensitive and simple liquid chromatographic determination of B6 vitamers, especially pyridoxal 5-phosphate. J Chromatogr A 722: 295–301.

    Article  CAS  Google Scholar 

  • Kok RM, Raster L, de Long APJM, Poll-The B, Saudubray JM, Jakobs C (1987) Stable isotope dilution analysis of pipecolic acid in cerebrospinal fluid, plasma, urine and amniotic fluid using electron capture negative ion mass fragmentography. Clin Chem Acta 168: 143–152.

    Article  CAS  Google Scholar 

  • Plecko B, Stöckler-Ipsiroglu S, Paschke E, Erwa W, Struys E, Jakobs C (2000) Pipecolic acid elevation in plasma and cerebrospinal fluid of two patients with pyridoxine-dependent epilepsy. Ann Neurol 48: 121–125.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatrics, University Hospital Graz, Austria

    B. Plecko

  2. Core Unit of Biomedical Research, Division of Laboratory and Animal Science and Genetics, Medical University Vienna, Austria

    H. Hoeger

  3. Medical Center, VU University, Amsterdam, The Netherlands

    C. Jakobs & E. Struys

  4. Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA

    C. Stromberger

  5. Clinical Department for Small Animals and Horses, Veterinary University Vienna, Austria

    M. Leschnik

  6. Department of Pediatrics, University Hospital Vienna, Austria

    A. Muehl & S. Stoeckler-Ipsiroglu

Authors
  1. B. Plecko
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. H. Hoeger
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. C. Jakobs
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. E. Struys
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. C. Stromberger
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. M. Leschnik
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. A. Muehl
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. S. Stoeckler-Ipsiroglu
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to B. Plecko.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Plecko, B., Hoeger, H., Jakobs, C. et al. Pipecolic acid concentrations in brain tissue of nutritionally pyridoxine-deficient rats. J Inherit Metab Dis 28, 689–693 (2005). https://doi.org/10.1007/s10545-005-0071-4

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10545-005-0071-4

Keywords

Search

Navigation