THE IDENTIFICATION OF A NINHYDRIN-POSITIVE URINARY COMPONENT RECENTLY REPORTED IN HYPOPHOSPHATASIA
In 1955, the occurrence of a hitherto unreported ninhydrin-positive substance in the urine and plasma of infants suffering from hypophosphatasia was described. Evidence published at that time and subsequently suggested that the material might be phosphorylethanolamine, but the isolation and unequivocal identification of the substance has not been reported.The present paper describes the isolation of a small amount of the previously unidentified compound from the urine of the heterozygous father of a seriously affected infant. The melting point, mixed melting point, X-ray diffraction pattern, infrared absorption spectrum, and N: P ratio establish the identity of the substance to be phosphorylethanolamine. Proof that this compound occurs in hypophosphatasia provides a logical basis for further study of the metabolic importance of this substance.