Tags: Disease.
Glycine encephalopathy (also known as non-ketotic hyperglycinemia or NKH) is a rare autosomal recessive disorder of glycine metabolism. After phenylketonuria glycine encephalopathy is the second most common disorder of amino acid metabolism. The disease is caused by defects in the glycine cleavage system an enzyme responsible for glycine catabolism. There are several forms of the disease with varying severity of symptoms and time of onset.
