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Variant non ketotic hyperglycinemia is caused by mutations in LIAS, BOLA3 and the novel gene GLRX5

This enzyme family also includes -mannosidases, and -galactosidases, which, like -glucuronidase, are predominantly encoded and produced by the gut microbiota 193,194

This cotranslational incorporation of Sec into selenoproteins presents significant problems to the cell which must recognize the UGA as a Sec codon rather than a STOP translation signal (10,11,12)

The synthesis of glucosinolates starts with the oxidation of the parent amino acid to an aldoxime, followed by the addition of a thiol group (through conjugation with glutathione) to produce thiohydroximate

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