LIAS and hyperinsulinemic hypoglycemia, familial, 4: However, since exogenous lipoic acid fails to rescue defects in cells derived from lipoylsynthase (LIAS)-deficient patients; Lias-deficiency in mice is embryo lethal and there is no substantial evidence to show that ACSM1 activates lipoic acid in vivo (Solmonson and DeBerardinis, 2018), this “scavenging pathway” may only be relevant for xenobiotics.