TY - CHAP M1 - Book, Section TI - Chapter 139. Disorders of Glycine, Serine, and Proline Metabolism A1 - Schwahn, Bernd Christian A2 - Rudolph, Colin D. A2 - Rudolph, Abraham M. A2 - Lister, George E. A2 - First, Lewis R. A2 - Gershon, Anne A. PY - 2011 T2 - Rudolph's Pediatrics, 22e AB - Glycine and serine are nonessential amino acids that feed into many synthetic pathways. Both are involved in transfer of one-carbon units. Proline is a nonessential amino acid that has unique biochemical properties. While most disorders of amino acid metabolism disrupt catabolic pathways, only a few affect the synthesis of amino acids. Three of them compromise the production of serine or proline. Formation and elimination of glycine occurs through many pathways. One precursor of glycine is choline, which can be oxidized to betaine and sequentially demethylated to dimethylglycine, sarcosine, and glycine, which is finally degraded to CO2 and NH3. Four known defects in glycine metabolism lead to distinct biochemical or clinical phenotypes. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/03/29 UR - accesspediatrics.mhmedical.com/content.aspx?aid=6724983 ER -