A novel 1-bp deletion in PITX3 causing congenital posterior polar cataract
2011

Mutation in PITX3 Causes Cataract

Sample size: 16 publication Evidence: moderate

Author Information

Author(s): Berry Vanita, Francis Peter J., Prescott Quincy, Waseem Naushin H., Moore Anthony T., Bhattacharya Shomi S.

Primary Institution: Institute of Ophthalmology, University College London

Hypothesis

Is there a genetic mutation in the PITX3 gene that causes congenital posterior polar cataract in a family?

Conclusion

The study identified a novel 1-bp deletion in the PITX3 gene that causes isolated posterior polar cataract.

Supporting Evidence

  • The mutation identified was a 1-bp deletion (542delC) in the PITX3 gene.
  • This mutation resulted in a frameshift in codon 181, likely producing an aberrant protein.
  • The mutation cosegregated with all affected family members.

Takeaway

A family with cataracts was studied, and researchers found a tiny change in a gene that likely caused the cataracts.

Methodology

Genome-wide linkage analysis and direct sequencing were used to identify the mutation in the PITX3 gene.

Participant Demographics

The study involved a four-generation English family with a total of 16 members.

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