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Related Experiment Videos

Patterning the heart's left-right axis: from zebrafish to man

A M Goldstein1, B S Ticho, M C Fishman

  • 1Cardiovascular Research Center, Massachusetts General Hospital, Boston, USA.

Developmental Genetics
|June 11, 1998
PubMed
Summary

Left-right asymmetry is crucial for vertebrate development. Disruptions in this process, particularly involving midline structures like the notochord, are linked to congenital heart and visceral anomalies.

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Area of Science:

  • Developmental Biology
  • Embryology
  • Medical Genetics

Background:

  • Normal left-right asymmetry is essential and conserved across vertebrates.
  • Errors in establishing this axis can result in congenital heart and abdominal visceral anomalies with severe clinical outcomes.

Purpose of the Study:

  • To review signaling mechanisms breaking left-right symmetry.
  • To explore the consequences of altered laterality signaling, including cardiac malformations.
  • To investigate the clinical association between midline structure anomalies and laterality defects.

Main Methods:

  • Review of signaling pathways involved in breaking left-right symmetry.
  • Analysis of congenital cardiac diseases linked to malpositions and malalignments.
  • Retrospective review of patients with laterality disturbances at Massachusetts General Hospital over 20 years.

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Main Results:

  • Left-right signal reversal or homogenization can lead to distinct consequences.
  • Specific malpositions and malalignments of cardiac chambers and outflow tracts are observed.
  • A significant association was found between laterality defects and anomalies of the spine and other midline structures.

Conclusions:

  • Midline structures, such as the notochord and floorplate, are crucial sources of laterality signals.
  • Disruptions in laterality signaling contribute to congenital anomalies.
  • Clinical findings support a strong link between midline defects and laterality disturbances.