Morphological analysis of corneal opacity in Shiba dog with GM1 gangliosidosis.
GM1 gangliosidosis is one of the inherited metabolic lysosomal storage disorders characterized by neurological symptoms caused by beta-galactosidase deficiency and consequent accumulation of GM1 ganglioside in neuronal cells. Shiba dogs affected with GM1 gangliosidosis have been found to suffer from corneal opacity. In our morphological analysis, keratocyte enlargement was induced by abnormal intracellular accumulation of neutral carbohydrates, resulting in the loss of normal arrangement of collagen fibrils in the opaque cornea was found to be associated with the disorder. We therefore conclude that corneal opacity in this Shiba dog with GM1 gangliosidosis may be caused by neutral carbohydrate accumulation in lysosomes, swelling and dysfunction of keratocytes, and subsequent irregular arrangement of collagen fibrils in the corneal proper substance.
Nagayasu
Aya
A
Department of Veterinary Anatomy, Rakuno Gakuen University, Hokkaido, Japan.
Nakamura
Taito
T
Yamato
Osamu
O
Tsuzuki
Keiko
K
Hosaka
Yoshinao
Y
Ueda
Hiromi
H
Tangkawattana
Prasarn
P
Takehana
Kazushige
K
D000818:Animals; D050260:Carbohydrate Metabolism / Q000502:physiology; D003094:Collagen / Q000648:ultrastructure; D003318:Corneal Opacity / Q000209:etiology / Q000473:pathology / Q000662:veterinary*; D004283:Dog Diseases / Q000473:pathology*; D004285:Dogs; D016537:Gangliosidosis, GM1 / Q000150:complications / Q000473:pathology / Q000662:veterinary*; D046529:Microscopy, Electron, Transmission / Q000662:veterinary
D003094:Collagen