In vivo evidence that Agxt2 can regulate plasma levels of dimethylarginines in mice

► Infusion of BAIB increased dimethylarginine plasma levels in mice. ► Infusion of BAIB decreased plasma levels of AGXT2-derived ADMA metabolites. ► Expression of Agxt2 was not affected by BAIB infusion. ► First in vivo evidence that endogenous Agxt2 regulates ADMA and SDMA plasma levels. ► Heritable hyper-BAIB-uria may be associated with impaired methylarginine metabolism.