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Reductions In Taurine Secondary To Photoreceptor Loss In Irish Setters With Rod-Cone Dysplasia, Gustavo D. Aguirre, S Y. Schmidt
Reductions In Taurine Secondary To Photoreceptor Loss In Irish Setters With Rod-Cone Dysplasia, Gustavo D. Aguirre, S Y. Schmidt
Gustavo D. Aguirre, VMD, PhD
These studies show that onset of photoreceptor cell degeneration preceded the loss of taurine in retinas of Irish setters with rod-cone dysplasia. The numbers of photoreceptor cell nuclei were within the normal adult range in affected setters at 10 through 26 days of age but declined rapidly between 26 and 45 days and more gradually thereafter; their numbers became reduced to 50% of normal at 45 days and then to 12-20% and 3-10% of normal at 192 and 346 days, respectively. Taurine concentrations increased within the photoreceptor cell layer during normal development to peak values (50 mM) at a time …
Disease Expression In Cultured Pigment Epithelium. Feline Mucopolysaccharidosis Vi., Lawrence E. Stramm, M Haskins, R J. Desnick, Gustavo D. Aguirre
Disease Expression In Cultured Pigment Epithelium. Feline Mucopolysaccharidosis Vi., Lawrence E. Stramm, M Haskins, R J. Desnick, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
Primary cultures of retinal pigment epithelial (RPE) cells from cats with mucopolysaccharidosis VI (MPS VI) have been initiated from mixed populations of cells (ie, derived from the entire eyecup and represented by both pigmented and nonpigmented RPE cells). The cells were enzymatically dissociated from the eyecup and seeded at 6 X 10(4) cells/cm2. Cells from normal and affected cats formed confluent monolayers of polygonal cells between 5-10 days in culture and maintained most of their in vivo morphologic characteristics. The only abnormality observed in the MPS VI-affected cultures was the accumulation of vacuolated intracytoplasmic inclusions; when numerous, these vacuoles caused …