Serum β-N-Acetylglucosaminidase in Patients with Symptomatic Porphyria

Abstract
Studies in 30 male patients with symptomatic porphyria reveal an increased activity in the serum of the lysosomal enzyme .beta.-N-acetylglucosaminidase (EC 3.2.1.30) as compared with a control group of 33 healthy males. Enzymatic activity is higher in the active stage of the disease (with fresh skin lesions) than in patients found in remission. A high degree of correlation was detected between the activity of .beta.-N-acetylglucosaminidase in the serum and uroporphyrin in the urine, and the total plasma porphyrins. The increased activity of this enzyme in the serum indicates that the lysosomes participate in the pathogenesis of this disorder.