Creatine metabolism in patients with urea cycle disorders.

ABSTRACT: The urea cycle generates arginine that is one of the major precursors for creatine biosynthesis. Here we evaluate levels of creatine and guanidinoacetate (the precursor in the synthesis of creatine) in plasma samples (n_s = 207) of patients (n_p = 73) with different types of urea cycle disorders (ornithine transcarbamylase deficiency (n_s = 22; n_p = 7), citrullinemia type 1 (n_s = 60; n_p = 22), argininosuccinic aciduria (n_s = 81; n_p = 31), arginase deficiency (n_s = 44; n_p = 13)). The concentration of plasma guanidinoacetate positively correlated (p < 0.001, R² = 0.64) with levels of arginine, but not with glycine in all patients with urea cycle defects, rising to leve

SUBMITTER: Ingoglia F 

PROVIDER: S-EPMC8387902 | biostudies-literature | 2021 Dec

REPOSITORIES: biostudies-literature