MOLECULE

17-Beta Hydroxysteroid Dehydrogenase III Deficiency

17β-Hydroxysteroid dehydrogenase III deficiency is a rare autosomal recessive disorder of sexual development condition that is a cause of 46,XY disorder of sex development. The impaired testosterone biosynthesis by 17β-hydroxysteroid dehydrogenase III, presents as atypical genitalia in affected males.