Mutations in the UBIAD1 Gene, Encoding a Potential Prenyltransferase, Are Causal for Schnyder Crystalline Corneal Dystrophy
2007

Mutations in the UBIAD1 Gene Cause Schnyder Crystalline Corneal Dystrophy

Sample size: 18 publication 10 minutes Evidence: high

Author Information

Author(s): Orr Andrew, Dubé Marie-Pierre, Marcadier Julien, Jiang Haiyan, Federico Antonio, George Stanley, Seamone Christopher, Andrews David, Dubord Paul, Holland Simon, Provost Sylvie, Mongrain Vanessa, Evans Susan, Higgins Brent, Bowman Sharen, Guernsey Duane, Samuels Mark

Primary Institution: Dalhousie University

Hypothesis

Is the UBIAD1 gene responsible for Schnyder crystalline corneal dystrophy?

Conclusion

The study identified five mutations in the UBIAD1 gene that are likely responsible for Schnyder crystalline corneal dystrophy.

Supporting Evidence

  • Five different mutations in the UBIAD1 gene were identified in affected families.
  • All mutations were found to segregate with the disease in the families studied.
  • None of the mutations were found in unaffected individuals or control samples.

Takeaway

This study found that a gene called UBIAD1 has changes that cause a rare eye disease that makes the cornea cloudy.

Methodology

The researchers performed linkage analysis and direct DNA sequencing on affected individuals from multiple families.

Potential Biases

Potential bias in participant selection as the study focused on specific families with the disease.

Limitations

The study may not account for all genetic variations due to the rarity of the disease.

Participant Demographics

Affected individuals were from a large multigenerational family in Nova Scotia and other small families of various ethnic backgrounds.

Statistical Information

P-Value

8.7

Statistical Significance

p<0.05

Digital Object Identifier (DOI)

10.1371/journal.pone.0000685

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