SuperMEN1: Pituitary, Parathyroid and Pancreas (Advances in Experimental Medicine and Biology (668))

The vast expansion in research on tumorigenesis has greatly increased our understanding of tumor development in patients with inherited endocrine tumor syndromes. This book provides an up-to-date summaryfrom clinical basics and latest follow-up guidelines to the most recent molecular findings in multiple endocrine neoplasia Type I syndrome. Articles have been assembled by acknowledged experts in their respective fields to provide current perspectives on the clinical and genetic backgroundsofthis syndrome and to review carefully the latest discoveries concerning the possible functions and interactions ofmenin, the protein encoded by the MEN1gene,including itspossible role incell cycle regulation,hematopoiesis,and bone development. The goal ofthe book is also to present the most recent findings and the broadest aspects of the role of menin in tumorigenesis of the endocrine glands involved in MEN I syndrome (pituitary, parathyroid, endocrine pancreas and adrenal). The connection between the basic experimental and clinical points of view are highlighted through a discussion on animal models, which explores the field in both an inspiring and questioning manner with a focus on areas that remain to be clarified. Our goal was to bring together clinicians and basic researchers who represent a wide range of interests in this particular field of endocrine oncology. Presentingacomprehensive and current overviewofbasic experimental and clinical findings, this book can bring us closer to understanding endocrine tumorigenesis in multiple endocrine neoplasia Type I. Katalin Balogh, MD, PhD Attita Patocs, MD, MSc, PhD v ABOUT THEEDITORS. . . "The vast expansion in research on tumorigenesis has greatly increased our understanding of tumor development in patients with inherited endocrine tumor syndromes. SuperMEN1, Pituitary, Parathyroid and Pancreas provides an up-to-date summary from clinical basics and latest follow-up guidelines to the most recent molecular findings in multiple endocrine neoplasia Type 1 syndrome. Articles have been assembled by acknowledged experts in their respective fields to provide current perspectives on the clinical and genetic backgrounds of this syndrome and to review carefully the latest discoveries concerning the possible functions and interactions of menin, the protein encoded by the MEN1 gene, including its possible role in cell cycle regulation, hematopoiesis, and bone development. The goal of this valume is also to present the most recent findings and the broadest aspects of the role of menin in tumorigenesis of the endocrine glands involved in MEN 1 syndrome (pituitary, parathyroid, endocrine pancreas and adrenal). The connection between the basic experimental and clinical points of view are highlighted through a discussion on animal models, which explores the field in both an inspiring and questioning manner with a focus on areas that remain to be clarified. Our goal was to bring together clinicians and basic researchers who represent a wide range of interests in this particular field of endocrine oncology. Presenting a comprehensive and current overview of basic experimental and clinical findings, this book can bring us closer to understanding endocrine tumorigenesis in multiple endocrine neoplasia Type 1"--Publisher's description The vast expansion in research on tumorigenesis has greatly increased our understanding of tumor development in patients with inherited endocrine tumor syndromes. This book provides an up-to-date summaryfrom clinical basics and latest follow-up guidelines to the most recent molecular findings in multiple endocrine neoplasia Type I syndrome. Articles have been assembled by acknowledged experts in their respective fields to provide current perspectives on the clinical and genetic backgroundsofthis syndrome and to review carefully the latest discoveries concerning the possible functions and interactions ofmenin, the protein encoded by the MEN1gene, including itspossible role incell cycle regulation, hematopoiesis, and bone development. The goal ofthe book is also to present the most recent findings and the broadest aspects of the role of menin in tumorigenesis of the endocrine glands involved in MEN I syndrome (pituitary, parathyroid, endocrine pancreas and adrenal). The connection between the basic experimental and clinical points of view are highlighted through a discussion on animal models, which explores the field in both an inspiring and questioning manner with a focus on areas that remain to be clarified. Our goal was to bring together clinicians and basic researchers who represent a wide range of interests in this particular field of endocrine oncology. Presentingacomprehensive and current overviewofbasic experimental and clinical findings, this book can bring us closer to understanding endocrine tumorigenesis in multiple endocrine neoplasia Type I. Katalin Balogh, MD, PhD Attita Patocs, MD, MSc, PhD v ABOUT THEEDITORS

The vast expansion in research on tumorigenesis has greatly increased our understanding of tumor development in patients with inherited endocrine tumor syndromes. SuperMEN1, Pituitary, Parathyroid and Pancreas provides an up-to-date summary from clinical basics and latest follow-up guidelines to the most recent molecular findings in multiple endocrine neoplasia Type 1 syndrome. Articles have been assembled by acknowledged experts in their respective fields to provide current perspectives on the clinical and genetic backgrounds of this syndrome and to review carefully the latest discoveries concerning the possible functions and interactions of menin, the protein encoded by the MEN1 gene, including its possible role in cell cycle regulation, hematopoiesis, and bone development. The goal of this valume is also to present the most recent findings and the broadest aspects of the role of menin in tumorigenesis of the endocrine glands involved in MEN 1 syndrome (pituitary, parathyroid, endocrine pancreas and adrenal). The connection between the basic experimental and clinical points of view are highlighted through a discussion on animal models, which explores the field in both an inspiring and questioning manner with a focus on areas that remain to be clarified. Our goal was to bring together clinicians and basic researchers who represent a wide range of interests in this particular field of endocrine oncology. Presenting a comprehensive and current overview of basic experimental and clinical findings, this book can bring us closer to understanding endocrine tumorigenesis in multiple endocrine neoplasia Type 1.

MEN1 clinical background / Peter Igaz Genetic background of MEN1 : from genetic homogeneity to functional diversity / Patrick Gaudray and Günther Weber Menin : the protein behind the MEN1 syndrome / Maria Papaconstantinou ... [et al.] Cellular functions of menin / Geoffrey N. Hendy, Hiroshi Kaji, and Lucie Canaff The role of menin in hematopoiesis / Ivan Maillard and Jay L. Hess Role of menin in bone development / Hiroshi Kaji, Lucie Canaff, and Geoffrey N. Hendy Activin, TGF-b, and menin in pituitary tumorigenesis / Jean-Jacques Lebrun The role of menin in parathyroid tumorigenesis / Colin Davenport and Amar Agha Role of menin in neuroendocrine tumorigenesis / Terry C. Lairmore and Herbert Chen Adrenal tumors in MEN1 syndrome and the role of menin in adrenal tumorigenesis / Attila Patocs, Katalin Balogh, and Karoly Racz Functional studies of menin through genetic manipulation of the MEN1 homolog in mice / Dheepa Balasubramanian and Peter C. Scacheri. Front Matter....Pages i-xvii MEN1 Clinical Background....Pages 1-15 Genetic Background of MEN1: From Genetic Homogeneity to Functional Diversity....Pages 17-26 Menin: The Protein Behind the MEN1 Syndrome....Pages 27-36 Cellular Functions of Menin....Pages 37-50 The Role of Menin in Hematopoiesis....Pages 51-57 Role of Menin in Bone Development....Pages 59-67 Activin, TGF-β and Menin in Pituitary Tumorigenesis....Pages 69-78 The Role of Menin in Parathyroid Tumorigenesis....Pages 79-86 Role of Menin in Neuroendocrine Tumorigenesis....Pages 87-95 Adrenal Tumors in MEN1 Syndrome and the Role of Menin in Adrenal Tumorigenesis....Pages 97-103 Functional Studies of Menin through Genetic Manipulation of the Men1 Homolog in Mice....Pages 105-115 Back Matter....Pages 117-118