Localization of an adenosine deaminase-binding protein in human kidney.

A protein which specifically complexes with adenosine deaminase (complexing protein) has been localized in human kidney. Thin sections of renal tissue were treated with rabbit anti-complexing protein serum followed by fluorescein-labeled goat anti-rabbit gamma globulin serum. Complexing protein was detected in the proximal tubules of four normal kidneys. The glomeruli in sections from one of the four normal kidneys were also positive. Lung, liver, spleen, cardiac muscle, skeletal muscle, and kidney medulla were negative by this technique. The glomeruli as well as the proximal renal tubules of two patients with combined immunodeficiency disease-adenosine deaminase deficiency and three of seven patients with kidney disease contained easily detectable levels of complexing protein.