title: Epithelial phenotype in total sclerocornea.
abstract: OBJECTIVE
  To understand whether the epithelial phenotype in total sclerocornea is corneal or conjunctival in origin.
  
  
  METHODS
  Four cases of total sclerocornea (male:female = 1:3; mean age = 5.4 ± 4.3; 1-11 years old) who received penetrating keratoplasty (PKP) at our hospital between 2008 and 2011 were included. Corneal buttons obtained during PKP were used for transmission electron microscopy (TEM) as well as immunoconfocal microscopy for cytokeratins 3, 12, and 13, goblet cell mucin MUC5AC, connexin 43, stem cell markers p63 and ABCG2, laminin-5, and fibronectin.
  
  
  RESULTS
  After a mean follow-up period of 38.8 ± 14.0 (12-54) months, the grafts remained clear in half of the patients. TEM examination revealed a markedly attenuated Bowman's layer in the scleralized corneas, with irregular and variably thinned collagen lamellar layers, and disorganization and random distribution of collagen fibrils, which were much larger in diameter compared with a normal cornea. Immunoconfocal microscopy showed that keratin 3 was expressed in all four patients, while p63, ABCG2, and MUC5AC were all absent. Cornea-specific keratin 12 was universally expressed in Patients 1 to 3, while mucosa (including conjunctiva)-specific keratin 13 was negative in these patients. Interestingly, keratin 12 and 13 were expressed in Patient 4 in a mutually exclusive manner. Linear expression of laminin-5 in the basement membrane zone and similar expression of fibronectin were observed.
  
  
  CONCLUSIONS
  The epithelia in total sclerocornea are essentially corneal in phenotype, but in the event of massive corneal angiogenesis, invasion by the conjunctival epithelium is possible.
authors.affiliation: Limbal Stem Cell Laboratory, Department of Ophthalmology, Chang Gung Memorial Hospital, Taoyuan, Taiwan ; Department of Chinese Medicine, College of Medicine, Chang Gung University, Taoyuan, Taiwan.
authors.forename: David Hui-Kang
authors.initials: DH
authors.lastname: Ma
authors.affiliation: Limbal Stem Cell Laboratory, Department of Ophthalmology, Chang Gung Memorial Hospital, Taoyuan, Taiwan.
authors.forename: Lung-Kung
authors.initials: LK
authors.lastname: Yeh
authors.affiliation: Limbal Stem Cell Laboratory, Department of Ophthalmology, Chang Gung Memorial Hospital, Taoyuan, Taiwan.
authors.forename: Hung-Chi
authors.initials: HC
authors.lastname: Chen
authors.affiliation: Department of Emergency Medicine, Oregan Health and Science University, Portland, OR.
authors.forename: Anna Marie
authors.initials: AM
authors.lastname: Chang
authors.affiliation: Limbal Stem Cell Laboratory, Department of Ophthalmology, Chang Gung Memorial Hospital, Taoyuan, Taiwan.
authors.forename: Yi-Ju
authors.initials: YJ
authors.lastname: Ho
authors.affiliation: Limbal Stem Cell Laboratory, Department of Ophthalmology, Chang Gung Memorial Hospital, Taoyuan, Taiwan.
authors.forename: Shirley H L
authors.initials: SH
authors.lastname: Chang
authors.affiliation: Department of Biology, University of California, Berkeley, CA.
authors.forename: Unique
authors.initials: U
authors.lastname: Yang
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018031:Connexin 43 / Q000378:metabolism; D003315:Cornea / Q000002:abnormalities* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D003316:Corneal Diseases / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D003710:Demography; D019573:Epithelium, Corneal / Q000378:metabolism / Q000473:pathology* / Q000601:surgery / Q000648:ultrastructure; D005260:Female; D006801:Humans; D007223:Infant; D007633:Keratins / Q000378:metabolism; D008297:Male; D010641:Phenotype
chemical_list: D018031:Connexin 43; D007633:Keratins
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