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10.1007/978-94-007-7893-1_8

http://scihub22266oqcxt.onion/10.1007/978-94-007-7893-1_8
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suck abstract from ncbi


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pmid24443024
      Adv+Exp+Med+Biol 2014 ; 802 (ä): 107-27
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  • Connective tissue disorders and cardiovascular complications: the indomitable role of transforming growth factor-beta signaling #MMPMID24443024
  • Wheeler JB ; Ikonomidis JS ; Jones JA
  • Adv Exp Med Biol 2014[]; 802 (ä): 107-27 PMID24443024 show ga
  • Marfan Syndrome (MFS) and Loeys-Dietz Syndrome (LDS) represent heritable connective tissue disorders that cosegregate with a similar pattern of cardiovascular defects (thoracic aortic aneurysm, mitral valve prolapse/regurgitation, and aortic root dilatation with regurgitation). This pattern of cardiovascular defects appears to be expressed along a spectrum of severity in many heritable connective tissue disorders and raises suspicion of a relationship between the normal development of connective tissues and the cardiovascular system. Given the evidence of increased transforming growth factor-beta (TGF-?) signaling in MFS and LDS, this signaling pathway may represent the common link in this relationship. To further explore this hypothetical link, this chapter will review the TGF-? signaling pathway, heritable connective tissue syndromes related to TGF-? receptor (TGFBR) mutations, and discuss the pathogenic contribution of TGF-? to these syndromes with a primary focus on the cardiovascular system.
  • |Adrenergic beta-Antagonists/therapeutic use [MESH]
  • |Angiotensin II Type 1 Receptor Blockers/therapeutic use [MESH]
  • |Antibodies, Neutralizing/pharmacology [MESH]
  • |Aortic Aneurysm, Thoracic/drug therapy/*genetics/pathology/surgery [MESH]
  • |Aortic Valve/pathology/surgery [MESH]
  • |Bicuspid Aortic Valve Disease [MESH]
  • |Gene Expression Regulation [MESH]
  • |Heart Defects, Congenital/drug therapy/*genetics/pathology/surgery [MESH]
  • |Heart Valve Diseases/drug therapy/*genetics/pathology/surgery [MESH]
  • |Humans [MESH]
  • |Loeys-Dietz Syndrome/drug therapy/*genetics/pathology/surgery [MESH]
  • |Marfan Syndrome/drug therapy/*genetics/pathology/surgery [MESH]
  • |Mutation [MESH]
  • |Receptors, Transforming Growth Factor beta/genetics [MESH]
  • |Signal Transduction/*genetics [MESH]
  • |Smad Proteins/genetics [MESH]


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