Gustavo A Aguirre

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Enzyme replacement therapy (ERT) has long been considered an approach to treating lysosomal storage disorders caused by deficiency of lysosomal enzymes. ERT is currently used to treat Gaucher disease and is being developed for several lysosomal storage disorders now that recombinant sources of the enzymes have become available. We have continued development(More)
Feline mucopolysaccharidosis VI (MPS VI) is a recessively inherited deficiency of arylsulfatase B (ASB). In the eye, the disease is expressed by the intracytoplasmic accumulation of vacuolated inclusions. These are present in connective tissue cells in the cornea, conjunctiva, sclera, choroid, and the stroma of the iris and ciliary body. In the iris and(More)
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(More)
Mucopolysaccharidosis (MPS) VI is due to a deficiency in the activity of N-acetylgalactosamine 4-sulfatase (4S), also known as arylsulfatase B. Previously, retroviral vector (RV)-mediated neonatal gene therapy reduced the clinical manifestations of MPS I and MPS VII in mice and dogs. However, sulfatases require post-translational modification by(More)
Differential interference contrast (Nomarski) optics and autoradiography were used to estimate independently the rate of outer segment renewal in the early stages of progressive rod-cone degeneration (PRCD) in dogs. We have found that significant reductions in renewal rate are demonstrable using the two methods of analysis: autoradiography = 34% reduction;(More)
The visual pigment of dogs affected with progressive rod-cone degeneration was compared with that of normal dogs. Absorption spectra from detergent extracts and from intact photoreceptors showed no significant difference between normal and affected animals in the shape of the absorption spectrum, the wavelength of maximum absorbance (gamma max = 506 nm), or(More)
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(More)
In the systemic mucopolysaccharidoses (MPS) in animals, corneal clouding resulted from storage of glycosaminoglycans (GAG) in stromal keratocytes. The corneal epithelium was normal (MPS VI and VII) or minimally affected (MPS I), and stromal edema was not a feature even though the corneal endothelium demonstrated variable pathology. The MPS I (cat) cornea(More)
Severe hypertension was diagnosed in a dog that initially was referred for evaluation of visual deficits and retinal hemorrhage and eventually was donated for medical treatment of hypertension. Initial blood pressure measured by direct methods was markedly high (systolic, 275 mm of Hg; diastolic, 170 mm of Hg). Measures of renal function were within normal(More)