hyperglycinuria
hyperglycinuria
[hi″per-gli″sin-u´re-ah]hy·per·gly·ci·nu·ri·a
(hī'pĕr-glī'si-nyū'rē-ă),hyperglycinuria
(hī′pər-glī′sə-no͝or′ē-ə)hyperglycinuria
A benign, inborn error of amino acid transport (OMIM:138500) characterised by excess of glycine in the urine, which may be associated with renal colic due to oxalate stones.Molecular pathology
Caused by defects of:
• SLC6A19, which encodes a protein that actively transports neutral amino acids, especially leucine, across the apical membrane of intestinal and renal epithelial cells;
• SLC6A20, which encodes a transporter that mediates the uptake of glycine as well as imino acids; and
• SLC36A2, which encodes a pH-dependent proton-coupled amino acid transporter that primarily transports small amino acids (e.g., glycine, alanine and proline).