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Essential role for ADAM19 in cardiovascular morphogenesis

Hong-Ming Zhou1, Gisela Weskamp, Valérie Chesneau

  • 1Cell Biology Program, Sloan-Kettering Institute, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.

Molecular and Cellular Biology
|December 16, 2003
PubMed

Insights

Mice lacking ADAM19 (a disintegrin and metalloprotease 19) showed severe congenital heart defects, including septal and valve abnormalities. This suggests ADAM19 mutations may cause human heart defects.

Area of Science:

  • Developmental Biology
  • Genetics
  • Cardiovascular Research

Background:

  • Congenital heart disease (CHD) is a prevalent birth defect with largely unknown causes.
  • Understanding the genetic basis of cardiac development is crucial for addressing CHD.

Purpose of the Study:

  • To investigate the role of ADAM19 (a disintegrin and metalloprotease 19) in cardiac morphogenesis.
  • To determine if ADAM19 deficiency leads to congenital heart defects in a mouse model.

Main Methods:

  • Generation and analysis of ADAM19-deficient mice (adam19-/-).
  • Detailed examination of cardiac structures and vasculature during embryonic development.
  • Assessment of ADAM19 expression patterns in developing tissues.

Main Results:

  • ADAM19-deficient mice exhibited severe cardiac defects, including ventricular septal defects (VSDs).
  • Abnormalities in aortic and pulmonic valve formation, leading to stenosis, were observed.
  • Cardiac vasculature development was also impaired in these mice.
  • ADAM19 expression was prominent in key cardiac developmental structures like the conotruncus and endocardial cushions.

Conclusions:

  • ADAM19 is essential for normal cardiac morphogenesis, particularly for septal and valve formation.
  • ADAM19 deficiency in mice results in severe, often lethal, congenital heart defects.
  • Mutations in ADAM19 represent a potential genetic cause for human congenital heart valve and septal defects.

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