Molecular biology of aortic diseases
Rev. Soc. Cardiol. Estado de Säo Paulo
;
11(6): 1033-1043, nov.-dez. 2001. ilus
Artigo
em Inglês
| LILACS
| ID: lil-391571
ABSTRACT
The elastic property of several tissues is conferred by the elastic fiber, composed of a homogeneous core of elastin surrounded by microfibrils. Defects in these components lead to different genetic diseases with important cardiovascular manifestations in the aorta. Here we will review these genetic disorders associated with pathologies of the aorta, focusing on the molecular defects underlying them, and on how the elucidation of the molecular lesion can lead to a better understanding of disease progression. In particular, we will discuss the recent advances in the molecular basis of supravalvar aortic stenosis, Williams syndrome, Marfan syndrome, and the fibrillinopathies.
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Índice:
LILACS (Américas)
Assunto principal:
Síndrome de Williams
/
Estenose Aórtica Supravalvular
/
Síndrome de Marfan
Limite:
Animais
/
Feminino
/
Humanos
/
Masculino
Idioma:
Inglês
Revista:
Rev. Soc. Cardiol. Estado de Säo Paulo
Assunto da revista:
Cardiologia
Ano de publicação:
2001
Tipo de documento:
Artigo
País de afiliação:
Brasil
Instituição/País de afiliação:
UNIFESP/BR
/
USP/BR
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