[Mutation analysis for GJB2 and LOR genes in two patients with Vohwinkel syndrome]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2013 Apr;30(2):203-6. doi: 10.3760/cma.j.issn.1003-9406.2013.04.018.
[Article in Chinese]

Abstract

Objective: To detect potential mutations of gap junction protein beta 2 (GJB2) and loricrin (LOR) genes in two patients with Vohwinkel syndrome.

Methods: Polymerase chain reaction and DNA sequencing were used for detecting potential mutations in the GJB2 and LOR genes. Parents of one patient and 50 healthy individuals were used as controls.

Results: A novel homozygous missense mutation (c.A796G) of LOR gene was detected in one patient. The same mutation was not found in the other patient, their relatives and the 50 healthy controls.

Conclusion: A missence mutation of LOR gene was detected in a patient with Vohwinkel syndrome.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple / genetics*
  • Abnormalities, Multiple / pathology
  • Adult
  • Child, Preschool
  • Connexin 26
  • Connexins / genetics*
  • Female
  • Hand Deformities, Congenital / genetics*
  • Hand Deformities, Congenital / pathology
  • Hearing Loss, Sensorineural / genetics*
  • Hearing Loss, Sensorineural / pathology
  • Humans
  • Keratoderma, Palmoplantar / genetics*
  • Keratoderma, Palmoplantar / pathology
  • Male
  • Membrane Proteins / genetics*
  • Mutation, Missense*
  • Sequence Analysis, DNA

Substances

  • Connexins
  • GJB2 protein, human
  • Membrane Proteins
  • loricrin
  • Connexin 26

Supplementary concepts

  • Vohwinkel syndrome