By A.L & Schwartz, Morton Latner
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Additional resources for Advances in Clinical Chemistry, Vol. 23
Alton-Mackey and Walker (A17) have shown that pups of vitamin B,-deficient dams show pronounced growth depression in the first 3 weeks of life, and even offspring of rats fed 100% of the recommended pyridoxine intakes during gestation grew less than controls fed 400%. Other effects noted in the offspring of rats made vitamin B, deficient during pregnancy or lactation include delay in onset of reflexes and advanced neuromotor coordination (A18), major malformations such as omphalocele, exencephaly, cleft palate, micrognathia, splenic hypoplasia (DIO), alterations in DNA content of the fetal brain (D11, D12), and impaired elongation of fatty acids in brain, leading to impaired myelination (C16).
Two pedigrees have been described in which the vitamin responsiveness was dose specific for each pedigree, and quite different pyridoxine intakes were required to satisfy the requirements of each pedigree (S14). This suggests a significant degree of genetic heterogeneity but, as the exact nature of the binding site on the apoenzyme has yet to be worked out, the significance of these observations is not clear. Recent work on lymphoid cell lines from cystathioninuric patients who respond to pyridoxine shows that the cells produced cystathionase molecules altered in their ability to combine with the vitamer, but with their antigenic identity unaltered.
Four patients who had been treated with a gluten-free diet showed plasma pyridoxal phosphate levels within the normal range. Reinken and his team (R8) investigated pyridoxal phosphate levels and the activity of pyridoxal kinase in the serum and duodenal mucosa of 14 children with acute celiac disease and of 10 children in clinical and biochemical remission; they found reduced levels of the vitamin in both plasma and duodenal mucosa of the acutely ill children, but with pyridoxal kinase levels significantly higher in mucosa of both groups of children with celiac disease than in normal controls.