Molecular screening of the CYP4V2 gene in Bietti crystalline dystrophy that is associated with choroidal neovascularization
2011

Genetic Study of Bietti Crystalline Dystrophy and Choroidal Neovascularization

Sample size: 3 publication Evidence: low

Author Information

Author(s): Mamatha Gandra, Umashankar Vetrivel, Kasinathan Nachiappan, Krishnan Tandava, Sathyabaarathi Ravichandran, Karthiyayini Thirumalai, Amali John, Rao Chetan, Madhavan Jagadeesan

Primary Institution: Vision Research Foundation, Sankara Nethralaya, Chennai, India

Hypothesis

What genetic variations are associated with Bietti crystalline dystrophy and its rare occurrence of choroidal neovascularization?

Conclusion

A novel mutation was identified in a patient with Bietti crystalline dystrophy who did not have choroidal neovascularization, while those with CNV did not show any pathogenic variations.

Supporting Evidence

  • Two patients with Bietti crystalline dystrophy had choroidal neovascularization.
  • A novel single base-pair duplication mutation was found in one patient without CNV.
  • No pathogenic variations were observed in the CYP4V2 gene in patients with CNV.

Takeaway

This study looked at three patients with a rare eye disease and found a new genetic mutation in one patient, but not in the others who had a related condition.

Methodology

Genomic DNA was isolated from blood leukocytes, and coding exons and flanking introns of CYP4V2 and exon 5 of TIMP3 were amplified and sequenced.

Limitations

The study had a small sample size and did not identify variations in the TIMP3 gene.

Participant Demographics

Three patients of Asian Indian origin with Bietti crystalline dystrophy.

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