Cell surface glycoconjugate abnormalities and corneal epithelial wound healing in the Pax6(+/-) mouse model of aniridia-related keratopathy

Romana Kucerova, Jingxing Ou, Diane Lawson, Lucy J. Leiper, J. Martin Collinson

Research output: Contribution to journalArticlepeer-review

11 Citations (Scopus)


PURPOSE. Congenital aniridia due to heterozygosity for Pax6 is associated with ocular surface disease, including keratopathy. This study investigated how defects in glycoconjugate component of the cell surface of Pax6(+/-) could cause the abnormal cellular migration phenotypes associated with the disease.

METHODS. Immunohistochemistry, lectin-based histochemistry, conventional staining techniques, and proteomic assays were performed on eyes and cultured corneal epithelial cells from wild-type and Pax6(+/-) littermates. Wild-type cells were manipulated in culture to replicate the glycoconjugate abnormalities found in Pax6 heterozygotes and determine the consequences for wound healing.

RESULTS. Multiple glycoconjugate defects were found in Pax6-mutant cells. Lectin cytochemistry of corneal epithelial cells suggested a partial failure of glycoprotein trafficking. Blocking cell surface carbohydrate moieties in wild-type corneal cells caused wound-healing delays similar to those seen in untreated Pax6(+/-) cells.

CONCLUSIONS. Alterations to the cell surface glycoconjugate signature of Pax6(+/-) corneal epithelia restrict the ability of cells to initiate migration in response to wounding. This underlies the observed wound-healing delay in cultured Pax6(+/-) epithelia.

Original languageEnglish
Pages (from-to)5276-5282
Number of pages7
JournalInvestigative Ophthalmology & Visual Science
Issue number12
Publication statusPublished - Dec 2006


  • metalloproteinase gelatinase B
  • heparan-sulfate proteoglycans
  • small-eye
  • expression
  • PAX6
  • gene
  • lens
  • differentiation
  • morphogenesis
  • regeneration

Cite this