Journal
FEBS LETTERS
Volume 586, Issue 20, Pages 3653-3657Publisher
WILEY
DOI: 10.1016/j.febslet.2012.08.020
Keywords
Homoarginine; Arginine:glycine amidinotransferase (AGAT); Argininosuccinate synthase (ASS); Cardiovascular disease
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Low plasma homoarginine has emerged as a risk marker for cardiovascular disease. We exploited cells of a patient with a rare inborn error of metabolism to explore potential pathways of homoarginine synthesis, using stable isotopes and mass spectrometry. Control lymphoblasts, as opposed to lymphoblasts from an arginine:glycine amidinotransferase (AGAT)-deficient patient, were able to synthesize homoarginine from arginine and lysine. In contrast, in a patient with a deficiency of the urea cycle enzyme argininosuccinate synthase, plasma homoarginine was not decreased. We conclude that promiscuous activity of AGAT, a key enzyme in creatine synthesis, plays a pivotal role in homoarginine synthesis. (C) 2012 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.
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