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Pyridoxine-responsive primary acquired sideroblastic anaemia : in vitro and in vivo effects of vitamin B6 on decreased 5-aminolaevulinate synthase activity

Meier, P. J. and Fehr, J. and Meyer, U. A.. (1982) Pyridoxine-responsive primary acquired sideroblastic anaemia : in vitro and in vivo effects of vitamin B6 on decreased 5-aminolaevulinate synthase activity. Scandinavian journal of haematology, Vol. 29, H. 5. S. 421-424.

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Official URL: http://edoc.unibas.ch/dok/A5261814

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Abstract

The activity of 5-aminolaevulinate (ALA) synthase, the first and rate-limiting of haem synthesis, was markedly reduced (13% of controls) in erythroblasts of a patient with acquired, primary sideroblastic anaemia (PASA). The reduced activity of ALA synthase could not be restored in vitro with 1 mmol/l pyridoxal-5-phosphate (PLP). Treatment of the patient with pyridoxine for several months increased the ALA synthase activity from 13% to 50% of controls in the absence and to 100% in the presence of PLP in the incubation medium. These studies suggest that both increased degradation of apo-ALA synthase and decreased affinity of ALA synthase for PLP may be involved in pyridoxine-responsive PASA.
Faculties and Departments:11 Rektorat und Verwaltung > Vizerektorat Forschung
UniBasel Contributors:Meier-Abt, Peter J.
Item Type:Article, refereed
Bibsysno:Link to catalogue
Publisher:Munksgaard
ISSN:0036-553X
Note:Publication type according to Uni Basel Research Database: Journal item
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Last Modified:22 Mar 2012 14:24
Deposited On:22 Mar 2012 13:40

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