c.194 A>C (Q65P) mutation in the LMX1B gene in patients with nail-patella syndrome associated with glaucoma
2011

Mutation in LMX1B Gene Linked to Nail-Patella Syndrome and Glaucoma

Sample size: 7 publication Evidence: moderate

Author Information

Author(s): Romero Pablo, Sanhueza Felipe, Lopez Pamela, Reyes Loreto, Herrera Luisa

Primary Institution: Departamento de Oftalmología, Hospital Clínico José Joaquín Aguirre, Universidad de Chile, Santiago, Chile

Hypothesis

Is there a connection between the LMX1B gene mutation and the expressivity of glaucoma in patients with nail-patella syndrome?

Conclusion

The c.194 A>C mutation in the LMX1B gene is associated with varying degrees of glaucoma in affected family members.

Supporting Evidence

  • Five family members were diagnosed with nail-patella syndrome, with three having open angle glaucoma.
  • The c.194 A>C mutation was found in all affected individuals but not in unaffected family members.
  • Normal renal function was observed in all subjects evaluated.

Takeaway

This study found a gene change in a family that causes a rare condition called nail-patella syndrome, which can lead to eye problems like glaucoma.

Methodology

The study involved genetic screening and clinical evaluations of affected and unaffected family members.

Limitations

The study is limited to a single family, which may not represent the broader population with nail-patella syndrome.

Participant Demographics

Five affected family members and two unaffected members were studied, spanning three generations.

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