Steroid Hormone Resistance, Softcover reprint of the original 1st ed. 1986 Mechanisms and Clinical Aspects Advances in Experimental Medicine and Biology Series, Vol. 196

This volume represents the first attempt to present in one place the clinical syndromes and the pathophysiologic basis for the "resistance states" to each of the classes of steroid hormones. Glucocorticoids, mineralocorticoids, androgens, estrogens, progesterone and vitamin D have widely diverse roles ranging from the control of homeostasis to reproduction and bone formation. They are similar in that they share a chemical structure and that their action is in the cell nucleus where they induce transcription of specific genes leading to synthesis of function-specific proteins. Clinical syndromes of steroid hormone resistance to androgens (complete and partial testicular feminization), aldosterone (pseudo­ hypoaldosteronism) and vitamin D (vitamin D-dependent rickets type II) have been known for many years. Progesterone and glucocorticoid resistance syndromes have been described only recently. Resistance to estrogens has not been reported in man or in animals. It is hoped that a detailed reexamination of what is known about each of these conditions at the clinical and molecular levels will enhance our understanding of the function of these hormones and their mechanisms of action. New insight and research initiatives should result. G.P. Chrousos D.L. Loriaus M.B. Lipsett vii ACKNOWLEDGMENTS The contents of this volume are based in part on the proceedings of an International Conference held in Bethesda in the summer of 1984. This conference was sponsored by the National Institute of Child Health and Human Development, Bethesda, Maryland.

Molecular Actions of Steroid Hormones.- Molecular Actions of Steroid Hormones.- Steroid Receptor Activation: The Glucocorticoid Receptor as a Model System.- “Defective” Receptors in Steroid-Resistant Conditions May Be Proteolytic Artifacts.- Characteristics and Specificity of the Glucocorticoid “Carrier” of Rat Liver Plasma Membrane.- Glucocorticoid Functions and Resistance.- Mechanisms of Glucocorticoid Hormone Action.- Glucocorticoid Physiology, Pharmacology and Stress.- Cortisol Resistance in Man.- Glucocorticoid Receptors and Glucocorticoid Resistance in Human Leukemia in Vivo and in Vitro.- The New World Primates as Animal Models of Glucocorticoid Resistance.- Mineralocorticoid Action and Resistance.- Aldosterone Receptors.- Models of Aldosterone Action on Sodium Transport: Emerging Concepts.- Pseudohypoaldosteronism: A Review and Report of Two New Cases.- Animal Models of Mineralocorticoid Resistance.- Androgen Action and Resistance.- Differential Regulation of Specific Gene Expression in Mouse Kidney by Androgens and Antiandrogens.- Androgen Receptors in Normal and Abnormal Male Sexual Differentiation.- Androgen Resistance in Man.- Animal Models of Androgen Insensitivity.- High Plasma Steroid Levels in the Squirrel Monkey: Deficient Receptors or Metabolism?.- Female Sex Steroids: New Aspects on the Mechanism of Action and Resistance States.- Progesterone Receptor Interaction in the 5’-Flanking Regulatory Region of the Ovalbumin Gene.- An Endogenous Ligand for Type II Binding Sites in Normal and Neoplastic Tissues.- Progesterone Resistance.- Estrogen and Antiestrogen Resistance in Human Breast Cancer Cell Lines.- Altered Estrogen Action in the Senescent Rat Uterus: A Model for Steroid Resistance During Aging.- Vitamin D Functions, Cellular Actions andResistant States.- The Metabolism and Functions of Vitamin D.- 1,25-Dihydroxyvitamin D3 Receptors: Altered Functional Domains Are Associated with Cellular Resistance to Vitamin D3.- Clinical Features of Hereditary Resistance to 1,25-Dihydroxyvitamin D (Hereditary Hypocalcemic Vitamin D Resistant Rickets Type II).- The Molecular Basis for Resistance to 1,25-Dihydroxyvitamin D: Studies in Cells Cultured from Patients with Hereditary Hypocalcemic 1,25(OH)2D3 Resistant Rickets.- The Common Marmoset as an Animal Model for Vitamin D-dependent Rickets, Type II.