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
Jun 1986
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 …
Reductions In Taurine Secondary To Photoreceptor Loss In Irish Setters With Rod-Cone Dysplasia, Gustavo D. Aguirre, S Y. Schmidt
Apr 1985
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
Jan 1985
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 …
Experimental Eye Research Volume 35, Issue 6, December 1982, Pages 625–642 Cover Image Retinal Degenerations In The Dog Iii Abnormal Cyclic Nucleotide Metabolism In Rod-Cone Dysplasia, Gustavo Aguirre, Debora Farber, Richard Lolley, Paul O'Brien, James Alligood, R Fletcher
Nov 1982
Experimental Eye Research Volume 35, Issue 6, December 1982, Pages 625–642 Cover Image Retinal Degenerations In The Dog Iii Abnormal Cyclic Nucleotide Metabolism In Rod-Cone Dysplasia, Gustavo Aguirre, Debora Farber, Richard Lolley, Paul O'Brien, James Alligood, R Fletcher
Gustavo D. Aguirre, VMD, PhD
In dogs bred to develop rod-cone dysplasia, retinal development is normal until 13 days of age.Afterwards, there is an arrest of visual cell differentiation. Rod inner segments remain diminutive and outer segments fail to elongate as in controls; the outer segments show lamellar disorientation and disorganization. Affected visual cells degenerate, but the degeneration process is more rapid and extensive for rods than cones.
Cyclic GMP levels become elevated in affected retinas early during the postnatal differentiation of visual cells; this elevation precedes any morphological evidence of photoreceptor disease. Retinal protein synthesis is normal during the time that retinal cGMP levels …
Pathogenesis Of Progressive Rod-Cone Degeneration In Miniature Poodles, Gustavo D. Aguirre, James P. Alligood, P O'Brien, N Buyukmihci
Oct 1982
Pathogenesis Of Progressive Rod-Cone Degeneration In Miniature Poodles, Gustavo D. Aguirre, James P. Alligood, P O'Brien, N Buyukmihci
Gustavo D. Aguirre, VMD, PhD
Visual cell pathologic changes and outer segment renewal were investigated in miniature poodles with progressive rod-cone degeneration. Early in this disease, visual cells in the posterior pole and equatorial regions show outer segment lamellar disorientation and vesicular profiles. Visual cells are normal in the periphery. Outer segment renewal determined after intravitreal injection of 3H-leucine was abnormally slower in affected animals than in controls. This renewal abnormality was similar in structurally normal and diseased photoreceptors, suggesting that the renewal defect is the earliest recognizable abnormality in the disease. The pigment epithelium was normal; the presence and density of pigment did not …
Pigmentary Abnormalities Of The Macula In Rhesus Monkeys: Clinical Observations, R W. Bellhorn, C D. King, Gustavo D. Aguirre, H Ripps, I M. Siegel, H C. Tsai
Nov 1981
Pigmentary Abnormalities Of The Macula In Rhesus Monkeys: Clinical Observations, R W. Bellhorn, C D. King, Gustavo D. Aguirre, H Ripps, I M. Siegel, H C. Tsai
Gustavo D. Aguirre, VMD, PhD
In a survey of 546 rhesus monkeys of various ages, 6.1% of the animals showed ophthalmoscopically visible hypopigmented spots in their maculas. There was a statistically significant correlation between the age of the animal and the degree of hypopigmentation. Electroretinographic responses and visually evoked potentials were evaluated in a selected group of monkeys with and without hypopigmented macular spots. No significant change in retinal function as a result of the macular abnormalities could be detected.
Gyrate Atrophy Of The Choroid And Retina In A Cat, D L. Valle, A P. Boison, P Jezyk, Gustavo D. Aguirre
Jan 1981
Gyrate Atrophy Of The Choroid And Retina In A Cat, D L. Valle, A P. Boison, P Jezyk, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
An adult male, domestic short-haired cat with generalized retinal atrophy was found to have a 60-fold increase in plasma ornithine and ornithinuria. Ornithine-8-aminotransferase activity was undetectable in its tissues and in its cultured skin fibroblasts. This feline condition is thus analogous to gyrate atrophy of the choroid and retina in humans.
Congenital Stationary Night Blindness: An Animal Model, D A. Witzel, R D. Wilson, E L. Smith, Gustavo D. Aguirre
Jul 1978
Congenital Stationary Night Blindness: An Animal Model, D A. Witzel, R D. Wilson, E L. Smith, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
Electroretinographic studies of myctalopic Appaloosa horses demonstrated photopic and scotopic abnormalities similar to those in humans with congenital stationary night blindness (CSNB) of the Schubert-Bornschein type. The phototopic abnormalities consisted of reduced b-wave amplitudes and slower than normal b-wave implict time. The dark-adapted ERG's consisted of a simple negative potential; the scotopic b-wave was nonrecordable. However, a normal c-wave was present in the dark-adapted response. Histologic studies demonstrated no structural abnormalities that could explain the functional defect.
The Electroretinogram In Dogs With Inherited Cone Degeneration, Gustavo D. Aguirre
Oct 1975
The Electroretinogram In Dogs With Inherited Cone Degeneration, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
The electroretinogram (ERG) of hemeralopic Alaskan malamute dogs contains only rod components. There is absence of the photopic b-wave which is normally elicited with red light stimuli during dark adaptation and, using flicker stimulation, only the first or rod branch of the flicker fusion response curve is present. At high stimulus intensity levels, the flicker response of hemeralopes is absent. A normal ERG is recorded from affected dogs using blue light stimuli and low intensity white light. In the adult hemeralope, the retina contains no cones.
Pathology Of Hemeralopia In The Alaskan Malamute Dog, Gustavo D. Aguirre
Feb 1974
Pathology Of Hemeralopia In The Alaskan Malamute Dog, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
A strain of Alaskan malamute dogs has been found to have day blindness (hemeralopia), a genetic anomaly transmitted as a simple autosomal recessive trait. The diagnosis was based on the absence of ophthalmoscopically visible abnormalities and the presence of complete blindness during daylight with return to visual function in dim light. These findings remained unchanged throughout the animal's life.
