Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=24443024
&cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests
in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215
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
(?): 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]
free
free
free
