Progress in Heritable Soft Connective Tissue Diseases -

Progress in Heritable Soft Connective Tissue Diseases (eBook)

Jaroslava Halper (Herausgeber)

eBook Download: PDF
2014 | 2014
VIII, 245 Seiten
Springer Netherland (Verlag)
978-94-007-7893-1 (ISBN)
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This volume is a reference handbook focusing on diseases like Marfan syndrome, Ehlers-Danlos syndrome, Loeys-Dietz syndrome and other heritable soft connective tissue diseases. The book presents detailed information for both basic scientists and for clinicians seeing patients. It is also a stepping stone for new investigations and studies that goes beyond the facts about the composition and biochemistry of the connective tissue and extracellular matrix, as the authors connect individual components to specific aspects of various soft tissue disorders and to the actual or potential treatment of them.

Progress in Heritable Soft Connective Tissue Diseases features very prominent physicians and scientists as contributors who bring their most recent discoveries to the benefit of readers. Their expertise will help clinicians with proper diagnosis of sometimes elusive and uncommon heritable diseases of soft connective tissues.

This book also offers an update on the pathophysiology of these diseases, including an emphasis on unifying aspects such as connections between embryonic development of the different types of connective tissues and systems, and the role of TGF-beta in development and physiology of soft tissues. This new set of data explains, at least in part, why many of these disorders are interconnected, though the primary pathophysiological events, such as gene mutations, may be different for each disorder.


This volume is a reference handbook focusing on diseases like Marfan syndrome, Ehlers-Danlos syndrome, Loeys-Dietz syndrome and other heritable soft connective tissue diseases. The book presents detailed information for both basic scientists and for clinicians seeing patients. It is also a stepping stone for new investigations and studies that goes beyond the facts about the composition and biochemistry of the connective tissue and extracellular matrix, as the authors connect individual components to specific aspects of various soft tissue disorders and to the actual or potential treatment of them.Progress in Heritable Soft Connective Tissue Diseases features very prominent physicians and scientists as contributors who bring their most recent discoveries to the benefit of readers. Their expertise will help clinicians with proper diagnosis of sometimes elusive and uncommon heritable diseases of soft connective tissues.This book also offers an update on the pathophysiology of these diseases, including an emphasis on unifying aspects such as connections between embryonic development of the different types of connective tissues and systems, and the role of TGF-beta in development and physiology of soft tissues. This new set of data explains, at least in part, why many of these disorders are interconnected, though the primary pathophysiological events, such as gene mutations, may be different for each disorder.

Chapter 1: Structure, physiology and biochemistry of collagens (Michael J. Mienaltowski and David E. Birk)Chapter 2: Basic components of connective tissues and extracellular matrix (Jaroslava Halper and Michael Kjaer)Chapter 3: Proteoglycans and diseases of soft tissues (Jaroslava Halper)Chapter 4: Advances in the use of growth factors for treatment of disorders of soft tissues (Jaroslava Halper)Chapter 5: Clinical, diagnostic, and therapeutic aspects of the Marfan syndrome (Jason R. Cook and Francesco Ramirez)Chapter 6: Loeys-Dietz syndrome (Lut Van Laer, Harry Dietz and Bart Loeys)Chapter 7: Connective Tissue Disorders and Cardiovascular Complications: The indomitable role of Transforming Growth Factor-beta signaling(Jason B. Wheeler, John S.konomidis, and Jeffrey A. Jones)Chapter 8: The Ehlers-Danlos Syndrome (Fransiska Malfait and Anne De Paepe)Chapter 9: Ehlers–Danlos syndrome associated with glycosaminoglycan abnormalities (Noriko Miyake, Tomoki Kosho, Naomichi Matsumoto)Chapter 10: Cutis laxa (Miski Mohamed, Michiel Voet, Thatjana Gardeitchik and Eva Morava)Chapter 11: Collagen type VI myopathies (Kate M.D. Bushby, James Collins and Debbie Hicks)Chapter 12: Mouse Models in Tendon and Ligament Research (Michael J. Mienaltowski and David E. Birk)Chapter 13: Connective tissue disorders in domestic animals (Jaroslava Halper)

Erscheint lt. Verlag 18.1.2014
Reihe/Serie Advances in Experimental Medicine and Biology
Advances in Experimental Medicine and Biology
Zusatzinfo VIII, 245 p. 35 illus., 31 illus. in color.
Verlagsort Dordrecht
Sprache englisch
Themenwelt Medizin / Pharmazie Medizinische Fachgebiete Innere Medizin
Studium 1. Studienabschnitt (Vorklinik) Biochemie / Molekularbiologie
Studium 2. Studienabschnitt (Klinik) Humangenetik
Naturwissenschaften Biologie Zellbiologie
Technik
Schlagworte aorta and cardiac valves • Ehlers-Danlos syndrome • Marfan syndrome • tendons and ligaments • Transforming Growth Factor beta
ISBN-10 94-007-7893-7 / 9400778937
ISBN-13 978-94-007-7893-1 / 9789400778931
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