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Tiglyl glycine is an amino acid conjugate and intermediate in the catabolism of the amino acid isoleucine.1 It is formed from tiglyl-CoA by glycine-N-acyltransferase. Urinary tiglyl glycine levels are increased in patients with β-ketothiolase deficiency, propionate metabolism disorders, or respiratory chain disorders.1,2
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1. Tiglylglycine excreted in urine in disorders of isoleucine metabolism and the respiratory chain measured by stable isotope dilution GC-
2. "Classical organic acidurias": Diagnosis and pathogenesis. Clin. Exp. Med. 17(3), 305-323 (2017).