Mutational screening of VSX1, SPARC, SOD1, LOX, and TIMP3 in keratoconus
2011

Genetic Factors in Keratoconus

Sample size: 302 publication Evidence: moderate

Author Information

Author(s): Patrizia De Bonis, Antonio Laborante, Costantina Pizzicoli, Raffaella Stallone, Raffaela Barbano, Costanza Longo, Emilio Mazzilli, Leopoldo Zelante, Luigi Bisceglia

Primary Institution: IRCCS Casa Sollievo della Sofferenza, San Giovanni Rotondo, Italy

Hypothesis

To evaluate the involvement of VSX1, SPARC, SOD1, LOX, and TIMP3 in keratoconus.

Conclusion

The study confirms the possible pathogenic role of VSX1 in keratoconus, while excluding LOX and TIMP3, and leaving the roles of SOD1 and SPARC unclear.

Supporting Evidence

  • A novel mutation p.G239R was found in VSX1.
  • Variants in SPARC and SOD1 were identified, but their pathogenic significance is unclear.
  • No pathogenic variants were found in LOX and TIMP3.
  • 225 sporadic and 77 familial keratoconus cases were analyzed.

Takeaway

This study looked at genes that might cause keratoconus, a condition that affects the eye, and found that one gene, VSX1, might be important, but more research is needed.

Methodology

Mutational analysis of five genes was performed by sequencing and fragment analysis in 302 patients diagnosed with keratoconus.

Limitations

The study does not definitively clarify the roles of SOD1 and SPARC in keratoconus.

Participant Demographics

302 unrelated probands from southern Italy, including 225 sporadic and 77 familial cases.

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