92 - Gender and Behavior in Subjects with Genetic Defects in Male Sexual Differentiation

Abstract

This chapter reviews male sexual differentiation in humans and enzyme defects in testosterone (T) biosynthesis, and action resulting in disorders of male sexual differentiation is also discussed. The syndrome of complete androgen insensitivity due to defects in the androgen receptor is addressed. The influence of sex steroids extends beyond their roles in sexual differentiation and development. In many animal species, hormones have been shown to be essential for sexual differentiation of the brain during development and for maintaining sexually dimorphic behavior throughout life. The principals of sex determination, genetic and hormonal, in humans have proven to be similar to other mammals. However, the hormonal influence on sexual dimorphic differences in the nervous system in humans and sex differences in behavior, and its correlation with those of other mammals is still an emerging field.