Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2015 Mar 11;10(3):e0120972.
doi: 10.1371/journal.pone.0120972. eCollection 2015.

Vitamin B6 in Plasma and Cerebrospinal Fluid of Children

Free PMC article

Vitamin B6 in Plasma and Cerebrospinal Fluid of Children

Monique Albersen et al. PLoS One. .
Free PMC article


Background: Over the past years, the essential role of vitamin B6 in brain development and functioning has been recognized and genetic metabolic disorders resulting in functional vitamin B6 deficiency have been identified. However, data on B6 vitamers in children are scarce.

Materials and methods: B6 vitamer concentrations in simultaneously sampled plasma and cerebrospinal fluid (CSF) of 70 children with intellectual disability were determined by ultra performance liquid chromatography-tandem mass spectrometry. For ethical reasons, CSF samples could not be obtained from healthy children. The influence of sex, age, epilepsy and treatment with anti-epileptic drugs, were investigated.

Results: The B6 vitamer composition of plasma (pyridoxal phosphate (PLP) > pyridoxic acid > pyridoxal (PL)) differed from that of CSF (PL > PLP > pyridoxic acid > pyridoxamine). Strong correlations were found for B6 vitamers in and between plasma and CSF. Treatment with anti-epileptic drugs resulted in decreased concentrations of PL and PLP in CSF.

Conclusion: We provide concentrations of all B6 vitamers in plasma and CSF of children with intellectual disability (±epilepsy), which can be used in the investigation of known and novel disorders associated with vitamin B6 metabolism as well as in monitoring of the biochemical effects of treatment with vitamin B6.

Conflict of interest statement

Competing Interests: The authors have declared that no competing interests exist.


Fig 1
Fig 1. Human vitamin B6 metabolism.
PDXK = pyridoxal kinase. PDXP = vitamin B6-specific phosphatase. PNPO = pyridox(am)ine phosphate oxidase.
Fig 2
Fig 2. A. Correlation (Spearman’s rho, p-value) between PLP and PL in plasma (ρ = 0.622, p<0.001) of children (n = 42).
B. Correlation (Spearman’s rho, p-value) between PL in CSF and PL in plasma (ρ = 0.806, p<0.001) of children (n = 35).
Fig 3
Fig 3. Concentrations of PL, PLP and PM in CSF of children using one or more AEDs (n = 51; from both the original and additional sets) compared to children not using any AEDs (n = 41).
Median concentrations of PL, PLP and PM were lower in CSF of children with AED-treated epilepsy than in CSF of children not using any AEDs.

Similar articles

See all similar articles

Cited by 7 articles

See all "Cited by" articles


    1. Mills PB, Surtees RA, Champion MP, Beesley CE, Dalton N, et al. Neonatal epileptic encephalopathy caused by mutations in the PNPO gene encoding pyridox(am)ine 5'-phosphate oxidase. Hum Mol Genet. 2005;14(8): 1077–86. - PubMed
    1. Mills PB, Struys E, Jakobs C, Plecko B, Baxter P, et al. Mutations in antiquitin in individuals with pyridoxine-dependent seizures. Nat Med. 2006;12(3): 307–9. - PubMed
    1. Waymire KG, Mahuren JD, Jaje JM, Guilarte TR, Coburn SP, et al. Mice lacking tissue non-specific alkaline phosphatase die from seizures due to defective metabolism of vitamin B-6. Nat Genet. 1995;11(1): 45–51. - PubMed
    1. Whyte MP, Mahuren JD, Fedde KN, Cole FS, McCabe ER, et al. Perinatal hypophosphatasia: tissue levels of vitamin B6 are unremarkable despite markedly increased circulating concentrations of pyridoxal-5'-phosphate. Evidence for an ectoenzyme role for tissue-nonspecific alkaline phosphatase. J Clin Invest. 1988;81(4): 1234–9. - PMC - PubMed
    1. Walker V, Mills GA, Peters SA, Merton WL. Fits, pyridoxine, and hyperprolinaemia type II. Arch Dis Child. 2000;82(3): 236–7. - PMC - PubMed

MeSH terms

Grant support

These authors have no support or funding to report.