Mutations in a novel serine protease PRSS56 in families with nanophthalmos
2011

Mutations in the PRSS56 Gene Linked to Nanophthalmos

Sample size: 3 publication Evidence: moderate

Author Information

Author(s): Orr Andrew, Dubé Marie-Pierre, Zenteno Juan C., Jiang Haiyan, Asselin Geraldine, Evans Susan C., Caqueret Aurore, Lakosha Hesham, Letourneau Louis, Marcadier Julien, Matsuoka Makoto, Macgillivray Christine, Nightingale Mathew, Papillon-Cavanagh Simon, Perry Scott, Provost Sylvie, Ludman Mark, Guernsey Duane L., Samuels Mark E.

Primary Institution: Dalhousie University

Hypothesis

Identifying additional genes for nanophthalmos will enhance understanding of eye growth regulation.

Conclusion

The PRSS56 gene is a strong candidate for causing nanophthalmos in the studied families.

Supporting Evidence

  • A linked locus on chromosome 2q37 was identified with a peak LOD score of 4.7.
  • Multiple segregating variants were found in the PRSS56 gene among affected families.
  • One family was homozygous for a pathogenic missense mutation in PRSS56.

Takeaway

Some people have very small eyes because of a gene called PRSS56, which can cause problems with vision.

Methodology

Families with nanophthalmos were studied using genetic linkage analysis and sequencing of candidate genes.

Limitations

The study was limited to a small number of families and may not represent all cases of nanophthalmos.

Participant Demographics

Families from eastern Canada and Mexico with a history of nanophthalmos.

Statistical Information

P-Value

p<0.05

Statistical Significance

p<0.05

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