Our way of diagnosing and treating inborn errors of the urea cycle enzymes is governed by how we conceive the functioning of the cycle. By the fact that the inherited enzyme deficiencies alter the normal conditions of the urea cycle, these inborn errors offer an opportunity to test our concepts of the regulation of the urea cycle. We have tried to simulate the urea cycle by computer models and test if the model is compatible with what we know from patients, assuming that concentration changes of metabolites in the liver would lead to alterations in the same direction in the body fluids, i.e., changes of amino acids in plasma or orotic acid as an indicator of carbamylphosphate [Bachmann, 1974,1980; Colombo, 1979] in the patients’ urine. Our aim was thus to test if our concept of how the urea cycle works is correct.

This content is only available via PDF.
1981
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.