Promiscuous activity of arginine:glycine amidinotransferase is responsible for the synthesis of the novel cardiovascular risk factor homoarginine.

Abstract:

: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.

journal_name

FEBS Lett

journal_title

FEBS letters

authors

Davids M,Ndika JD,Salomons GS,Blom HJ,Teerlink T

doi

10.1016/j.febslet.2012.08.020

subject

Has Abstract

pub_date

2012-10-19 00:00:00

pages

3653-7

issue

20

eissn

0014-5793

issn

1873-3468

pii

S0014-5793(12)00677-1

journal_volume

586

pub_type

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