Anterior segment mesenchymal dysgenesis in a large Australian family is associated with the recurrent 17 bp duplication in PITX3
2008

Study of Eye Development Issues in an Australian Family

Sample size: 21 publication Evidence: moderate

Author Information

Author(s): Kim M. Summers, Stephen J. Withers, Glen A. Gole, Sara Piras, Peter J. Taylor

Primary Institution: The Roslin Institute, The University of Edinburgh

Hypothesis

Does a recurrent 17 bp duplication in the PITX3 gene cause anterior segment mesenchymal dysgenesis (ASMD) in a large Australian family?

Conclusion

The study found that the same genetic duplication can lead to varying degrees of eye problems, from severe blindness to treatable cataracts.

Supporting Evidence

  • All clinically affected family members had the same 17 bp duplication of PITX3.
  • Prenatal diagnosis showed one fetus carried the duplication while another was normal.
  • The study identified a second family with the same genetic mutation.

Takeaway

Some people in the same family can have different eye problems because of a specific gene change, which can cause serious issues for some and minor ones for others.

Methodology

Clinical examinations and a PCR-based test for the PITX3 duplication were performed on 21 family members.

Limitations

The study does not clarify the reasons for the variability in symptoms among affected individuals.

Participant Demographics

The study involved a large Australian family with a history of ASMD and cataracts.

Want to read the original?

Access the complete publication on the publisher's website

View Original Publication