Abstract
Extract: The intracellular content and proportional distribution of B12 (cobalamin) derivatives in fibroblasts cultured from patients with various forms of methylmalonic aciduria, as well as from normal control subjects, has been determined by a two-dimensional chromatobioautographic technique. Each line of fibroblasts was grown in the presence of four concentrations of cobalamin, ranging from the 0.04–0.07 pmol/ml contained in the basal medium to 74 pmol/ml (100 ng/ml), added in form of hydroxocobalamin (OHCBI). Control cells grown in the basal medium contained substantial proportions of both methylcobalamin (MeCbl) and adenosyl-cobalamin (AdoCbl), with the former predominating. As increasing concentrations of OH-CBI were added to the growth medium, the total cellular cobalamin content increased without marked changes in the relative proportions of MeCbl, AdoCbl, and OHCbl. Three different patterns were discernable in the cobalamin distributions of the cells cultured from patients with methylmalonic aciduria (Table 1 and Fig. 1).
Speculation: Information about the cobalamin contents of cultured fibroblasts yields further understanding of cobalamin metabolism. Such information, obtained by the technique of two-dimensional chromatobioautography, permits definition of genetically determined abnormalities in the handling of cobalamins, as well as examination of possible correlations between cellular responses to increased concentrations of these compounds and the effectiveness of cobalamins in the clinical treatment of patients.
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Linnell, J., Matthews, D., Mudd, S. et al. Cobalamins in Fibroblasts Cultured from Normal Control Subjects and Patients with Methylmalonic Aciduria. Pediatr Res 10, 179–183 (1976). https://doi.org/10.1203/00006450-197603000-00007
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DOI: https://doi.org/10.1203/00006450-197603000-00007
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