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Arylsulfatase B Activity In Cultured Retinal Pigment Epithelium: Regional Studies In Feline Mucopolysaccharidosis Vi., Lawrence E. Stramm, R J. Desnick, M Haskins, Gustavo D. Aguirre
Arylsulfatase B Activity In Cultured Retinal Pigment Epithelium: Regional Studies In Feline Mucopolysaccharidosis Vi., Lawrence E. Stramm, R J. Desnick, M Haskins, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
Feline mucopolysaccharidosis VI (MPS VI) is a recessively inherited lysosomal storage disease resulting from a deficiency of arylsulfatase B (ASB). Previous histopathologic findings have indicated that the disease is expressed morphologically in non-pigmented retinal pigment epithelial cells (RPE) in the posterior pole and superior equatorial regions by the accumulation of vacuolated inclusions and eventual cellular hypertrophy, while pigmented regions in the periphery are minimally affected. To determine if the regional and age-dependent variations in disease severity result from differences in residual enzyme activity, primary cultures of feline MPS VI-affected RPE were initiated from defined regions of the eye and maintained …