GLYCYLPROLINE PEPTIDURIA IN FAMILIAL HYPEROSTOSIS OF OBSCURE NATURE

Two sisters with striking skeletal deformities, glycylproline peptiduria and elevated total urinary excretion of hydroxyproline were studied. Their pedigree suggested recessive inheritance of the disorder. The data indicated that these girls are further examples of an inherited disorder of bone metabolism in which glycylproline excretion may be the marker of a specific defect in collagen metabolism. Glycylprolinuria and hydroxyprolinuria reflect independent phenomena in collagen metabolism. Excretory patterns of imino acid containing peptides may provide the means to define the individual metabolic disorders of collagen. © 1969.