Familial iminoglycinuria

Iminoglycinuria associated with congenital nerve deafness Renal tubular defect glycine, proline, hydroxyproline Autosomal Rosenberg, L.E., Durant, J.L., and Elsas, L.J. II. Familial iminoglycinuria an inborn error of renal tubular transport. N. Engl. J. Med. 278, 1407-1413 (1968)... 

An amino acid found in elevated amounts in blood and urine in the inborn errors of metabolism, non ketotic hyperglycinaemia, (which is possibly due to a deficiency in the enzyme glycine decarboxylase) and propionic acidaemia (ketotic hyperglycinaemia). It is also found in increased amounts in urine along with proline and hydroxyproline in another inborn error, familial iminoglycinuria. This is due to defective membrane transport of these amino acids. 

Familial iminoglycinuria is an inborn error in which there is increased excretion of proline, hydroxyproline and glycine in the urine. It is due to defective renal tubular transport of these... 

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