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Mol Genet Metab. 2010;100 Suppl 1:S37-41. doi: 10.1016/j.ymgme.2010.02.017. Epub 2010 Feb 26.

Measuring in vivo ureagenesis with stable isotopes.

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  • 1Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA. yudkoff@email.chop.edu

Abstract

Stable isotopes have been an invaluable adjunct to biomedical research for more than 70years. Indeed, the isotopic approach has revolutionized our understanding of metabolism, revealing it to be an intensely dynamic process characterized by an unending cycle of synthesis and degradation. Isotopic studies have taught us that the urea cycle is intrinsic to such dynamism, since it affords a capacious mechanism by which to eliminate waste nitrogen when rates of protein degradation (or dietary protein intake) are especially high. Isotopes have enabled an appreciation of the degree to which ureagenesis is compromised in patients with urea cycle defects. Indeed, isotopic studies of urea cycle flux correlate well with the severity of cognitive impairment in these patients. Finally, the use of isotopes affords an ideal tool with which to gauge the efficacy of therapeutic interventions to augment residual flux through the cycle.

Copyright 2010 Elsevier Inc. All rights reserved.

PMID:
20338795
[PubMed - indexed for MEDLINE]
PMCID:
PMC2858793
Free PMC Article

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