Prolidase deficiency

T12. Tanoue, A., Endo, F and Matsuda, I., Structural organization of the gene for human prolidase (peptidase D) and demonstration of a partial gene deletion in a patient with prolidase deficiency. J. Biol. Chem. 265,11306-11311 (1990). 

The AAA thus has two photometers in series. Since for every eluting amino acid both signals are recorded, the so-called 570/440 absorbance ratio may be of help in the identification process. As an example the simple primary amino acids have a 570/440 ratio of approximately 6, whereas the sulfur amino acids (cystine, sulfocysteine) have much lower ratios, approaching a value of 1. Small peptides and glycyl-amino acids will react with ninhydrin, an important fact for the diagnosis of prolidase deficiency and aspartylglycosaminuria. 

Lombeck I, Wendel U, Versieck J, van Ballenberghe L, Bremer HJ, Duran R, Wadman S. Increased manganese content and reduced arginase activity in erythrocytes of a patient with prolidase deficiency (iminodipeptiduria), Eur J Pediatr 1986 144 571-3. 

Lopes I, Marques L, Neves E, SUva A, Taveira M, Pena R et al. Prolidase deficiency with hyperimmunoglob-ulin E a case report. Pediatr AUergy Immunol 2002 13 140-2. 

Prolidase deficiency Erythrocytes, leukocytes, 19pl3.2 fibroblasts 170100... 

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