[Biochemical characterization of a new variant of glucose-6-phosphate dehydrogenase (G-6-PD) deficiency with favism: G-6-PD Bielefeld (author's transl)]

Abstract

A new variant of G-6-PD with favism has been detected in a German family. After partial purification the enzyme was biochemically characterized. It revealed normal electrophoretic mobility, normal thermostability and a flat pH-activity curve. The Km-value for G-6-P was reduced, whereas this value was normal for NADP. The substrate analogues 2-desoxy G-6-P, Gal-6-p and deamino-NADP were utilized to increased rates. The biochemical data were compatible with the benign clinical course.