The exponential growth in our knowledge on the pathophysiology of hypothalamic-pituitary-thyroid axis and, in particular, of thyroid hormone action, largely stems from the studies on the various syndromes of resistance to the hormones involved in the function of the axis. These syndromes essentially encompass two forms, i. e. the resistance to thyroid hormones and that to TSH action. The contribution of molecular biology has been fundamental in understanding the etiology of these different thyroid disorders and led to the discovery of new pathogenetic mechanisms, e. g. the dominant negative effects of a mutant protein on the normal one. In this book, I have assembled an internationally renewed group of experts devoted to the studies of both basic and clinical thyroidology. Taking the syndromes of resistance as a template, the novelties on the mechanisms of action of the various hormones, the etiopathogenesis of the syndromes, the instruments for the diagnosis, as well as the pitfalls in managing these diseases, are deeply revisited. The insights from different animal models are discussed, with particular focus on tissue specific action of thyroid hormones, as well as on the still enigmatic mechanism of negative feedback control of thyroid hormones on TSH secretion and the different etiologic mechanisms causing thyroid hormone resistance in patients without mutations in the thyroid hormone receptor ~. The role of the structure of thyroid hormone and TSH receptors in designing agonist or antagonist molecules is extensively debated. Moreover, new forms of congenital or acquired resistance to thyroid hormones are presented.
Erscheinungsdatum: 12.12.2012

lgrsnf/A:\Springer\bok%3A978-1-4020-7852-1.pdf

nexusstc/Syndromes of Hormone Resistance on the Hypothalamic-Pituitary-Thyroid Axis/1741178803666cf5af28967fe8f03442.pdf

zlib/Medicine/Paul M. Yen M. D. (auth.), Paolo Beck-Peccoz (eds.)/Syndromes of Hormone Resistance on the Hypothalamic-Pituitary-Thyroid Axis_2245557.pdf

edited by Paolo Beck-Peccoz

Beck-peccoz, P.

Springer

Kluwer Academic Publishers

Springer Nature (Textbooks & Major Reference Works), New York, NY, 2012

Softcover reprint of the original 1st ed. 2004, PS, 2012

Endocrine updates -- 22, Boston, Massachusetts, 2004

Endocrine updates, 1st ed. 2004, New York, NY, 2004

United States, United States of America

Dec 12, 2012

lg1076592

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Includes bibliographical references and index.

Source title: Syndromes of Hormone Resistance on the Hypothalamic-Pituitary-Thyroid Axis (Endocrine Updates (22))

Front Matter....Pages i-vii
Molecular Basis of Thyroid Hormone Action....Pages 1-11
Tissues Specific Action of Thyroid Hormones: Insights from Knock out Animal Models....Pages 13-33
Learning from Nature’s Experiments on the Thyroid Hormone Receptor; X-Ray Structures of RTH Mutant Ligand-Binding Domains....Pages 35-56
Molecular Pathogenesis of Resistance to Thyroid Hormone....Pages 57-68
Syndromes of Resistance to Thyroid Hormone: Clinical Aspects....Pages 69-88
Resistance to Thyroid Hormone in the Absence of Mutations in the Thyroid Hormone Receptor Genes....Pages 89-107
Lessons Learned from TR-β Mutant Mice....Pages 109-118
Towards the Rational Design of Hormone Analogs Which Complement Receptor Mutations....Pages 119-136
Multi-Factorial Regulation of in Vivo Action of TRβ Mutants. Lessons Learned from RTH Mice with a Targeted Mutation in the TRβ Gene....Pages 137-148
The Effect of Vitamin A, Retinoids and Retinoid Receptors on the Hypothalamic-Pituitary-Thyroid Axis....Pages 149-163
Pituitary Resistance to Thyroxine Action Due to a Defect in the Type 2 Deiodinase....Pages 165-176
Different forms of Resistance to Thyrotropin (TSH) Action....Pages 177-191
Back Matter....Pages 193-197

This volume covers the most recent scientific findings of the syndromes of hormone resistance in the HPT axis, i.e., thyroid hormone resistance and resistance to TSH action. Mechanisms of action of the various hormones, etiopathology of the syndromes, instruments for diagnosis, as well as the difficulties in managing these diseases are covered. In general, these disorders of HPT axis are uncommon and thus difficult to diagnose and treat. As a result, this book is a useful resource to clinicians and helps delineate the clinical flow-chart for both diagnosis and treatment

Truly unique cutting edge state of the art information on basic and clinical research. A great help for the clinician to diagnose and treat this uncommon hormone resistance. Basic scientists learn how much has been discovered on the mechanism of hormone action from the mutations of the various genes involved to thyroid hormones and TSH.

2013-12-12