Defective adenosylcobalamin synthesis in a case of transcobalamin II deficiency |
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| Authors: | J. C. Linnell E. V. Quadros P. G. Elliott P. Malleson |
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| Affiliation: | (1) Department of Experimental Chemical Pathology, Vincent Square Laboratories of Westminster Hospital, 124 Vauxhall Bridge Road, SW1V 2RH London;(2) Department of Physics, Westminster Hospital, SW1P 2AP London;(3) Hospital for Sick Children, Great Ormond Street, WC1N 1EH London |
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| Abstract: | Cobalamin metabolism has been investigated in a new case of transcobalamin II (TC II) deficiency. Using the chromatobioautographic technique, an abnormal distribution of cobalamins was detected in the child's erythrocytes and reduced synthesis of adenosylcobalamin but not of methylcobalamin in cultured fibroblasts. These results suggest that there may be a close link between TC II-mediated cobalamin transport and intracellular synthesis of adenosylcobalamin (Ado-Cbl). Financial support from the Wellcome Trust is gratefully acknowledged. |
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