Reciprocal corneal transplantation fails to correct mucopolysaccharidosis VI corneal storage.
This report contains the results of studies designed to evaluate corneal clearing in mucopolysaccharidosis VI (MPS VI)-affected cats. Corneal buttons from affected cats were transplanted into normal cat corneas and, as controls, normal-to-normal and normal-to-affected transplants also were done. No clearing of the MPS VI graft or host beds occurred, nor was there any clouding of the normal donor or recipient corneal tissues. This assessment was made by serial clinical examinations over a 14-30 mo period and by light and electron microscopic examination of the corneal tissues at the end of the study. Lack of corneal clearing under conditions that would maximize such a process in this animal model indicates that corneal clearing is not an appropriate index for measuring the success of systemic therapy in MPS VI.
Aguirre
G
G
Scheie Eye Institute, School of Veterinary Medicine, University of Pennsylvania, Philadelphia 19104.
Raber
I
I
Yanoff
M
M
Haskins
M
M
D000818:Animals; D002415:Cats; D003316:Corneal Diseases / Q000473:pathology*; D003319:Corneal Stroma / Q000473:pathology / Q000648:ultrastructure; D016039:Corneal Transplantation / Q000473:pathology*; D004195:Disease Models, Animal; D009087:Mucopolysaccharidosis VI / Q000473:pathology*