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Full-Text Articles in Medicine and Health Sciences

Β-Glucuronidase Mediated Pathway Essential For Retinal Pigment Epithelial Degradation Of Glycosaminoglycans. Disease Expression And In Vitro Disease Correction Using Retroviral Mediated Cdna Transfer, Lawrence Stramm, John Wolfe, Edward Schuchman, Mark Haskins, Donald Patterson, Gustavo Aguirre Apr 1990

Β-Glucuronidase Mediated Pathway Essential For Retinal Pigment Epithelial Degradation Of Glycosaminoglycans. Disease Expression And In Vitro Disease Correction Using Retroviral Mediated Cdna Transfer, Lawrence Stramm, John Wolfe, Edward Schuchman, Mark Haskins, Donald Patterson, Gustavo Aguirre

Gustavo D. Aguirre, VMD, PhD

A β-glucuronidase mediated pathway for the degradation of glycosaminoglycans is present in the retinal pigment epithelium. The pathway has been defined using ocular tissues and cultured cells from mutant animals having a recessively inherited deficiency of the lysosomal enzyme. In situ, storage products accumulate in secondary lysosomes of the retinal pigment epithelium, the cytoplasm fills with inclusions and the cells hypertrophy; severity of the disease increases with aging. Deficient activity of β-glucuronidase is present in primary and second passage cultures. Radiolabel studies with 35SO4 show a significant retention of cell layer label by mutant retinal pigment epithelial cells …


Segregation Distortion In Inheritance Of Progressive Rod Cone Degeneration (Prcd) In Miniature Poodle Dogs, Gregory M. Acland, Susan Halloran-Blanton, Joann A. Boughman, Gustavo D. Aguirre Feb 1990

Segregation Distortion In Inheritance Of Progressive Rod Cone Degeneration (Prcd) In Miniature Poodle Dogs, Gregory M. Acland, Susan Halloran-Blanton, Joann A. Boughman, Gustavo D. Aguirre

Gustavo D. Aguirre, VMD, PhD

Segregation distortion was observed in inheritance of progressive rod–cone degeneration (prcd) in a colony of Miniature Poodle dogs. Breeding results, from both retrospective records and prospectively planned matings, were classified into five mating types: (1) affected to affected, (2) homozygous normal sire to any dam, (3) heterozygous to heterozygous, (4) heterozygous sire to affected dam, and (5) affected sire to heterozygous dam. For all but the last category, results were in accord with mendelian expectations for autosomal-recessive inheritance. However, litters of mating type 5 had fewer affected pups (20/77) than expected. The observed segregation ratio for this mating type (0.26) …


Evaluation Of Chicken Heterophil Adherence, Claire B. Andreasen, Kenneth S. Latimer, W. L. Steffens Jan 1990

Evaluation Of Chicken Heterophil Adherence, Claire B. Andreasen, Kenneth S. Latimer, W. L. Steffens

Claire B. Andreasen

Adherence of chicken heterophils was evaluated at 37 C using preconstructed columns containing various weights of nylon fiber (75 mg, 100 mg, or 125 mg) and whole blood anticoagulated with sodium heparin or 10% disodium ethylenediamine tetraacetic acid (EDTA). Additionally, 50-mg and 75-mg nylon fiber columns incubated at 41 C were used to evaluate heterophil adherence at an increased temperature. The mean percent adherence for heparin-anticoagulated blood applied to 75-mg, 100-mg, and 125-mg nylon fiber columns at 37 C was 76%, 92% and 97.4%, respectively. Samples applied to 50-mg and 75-mg columns at 41 C had adherence values of 27% …


Organ Hypertrophy And Responses Of Colon Microbial Populations Of Growing Swine To High Dietary Protein, James S. Dickson, Wilson G. Pond, Vincent H. Varel, Friday O. I. Anugwa, Gary S. Ross Jan 1990

Organ Hypertrophy And Responses Of Colon Microbial Populations Of Growing Swine To High Dietary Protein, James S. Dickson, Wilson G. Pond, Vincent H. Varel, Friday O. I. Anugwa, Gary S. Ross

James S. Dickson

Thirty-two castrated male crossbred growing pigs (average initial wt 26.9 kg) were used to determine the effect of a high level of dietary protein (37%) compared with a normal level of protein (15%) on enterobacteria and Campylobactersp. inhabitation in the large intestine and on visceral organ hypertrophy and the interrelationships between these two factors. Pigs were kept in pairs (eight pens of two pigs/diet) and fed their respective diets ad libitum. Eight pigs (two pens of two pigs fed each diet) were killed at wk 4, 8. 12 and 16 without fasting. Fecal samples were obtained every 2 wk from …


Metabolic Labeling Of Rod Outer Segment Phospholipids In Miniature Poodles With Progressive Rod-Cone Degeneration (Prcd), Mary Wetzel, Christian Fahlman, Paul O'Brien, Gustavo Aguirre Dec 1989

Metabolic Labeling Of Rod Outer Segment Phospholipids In Miniature Poodles With Progressive Rod-Cone Degeneration (Prcd), Mary Wetzel, Christian Fahlman, Paul O'Brien, Gustavo Aguirre

Gustavo D. Aguirre, VMD, PhD

The recessive genetic defect in miniature poodles which results in progressive rod-cone degeneration (prcd) has been investigated in an attempt to determine the biochemical abnormality involved. In the present study, the rod outer segments of young prcd affected miniature poodles and normal dogs have been compared with respect to the incorporation of intravitreally injected [3H]palmitic acid, [14C]linolenic acid, and [14C]docosahexaenoic acid into neutral lipids and phospholipids as well as [3H]palmitate and [14C]leucine into rhodopsin. In addition, 3 mm trephined punches of retinas were incubated with [ …


Frequency Of The Codon 807 Mutation In The Cgmp Phosphodiesterase Β-Subunit Gene In Irish Setters And Other Dog Breeds With Hereditary Retinal Degeneration, Gustavo D. Aguirre, V Baldwin, K M. Weeks, Gregory M. Acland, Kunal Ray Dec 1989

Frequency Of The Codon 807 Mutation In The Cgmp Phosphodiesterase Β-Subunit Gene In Irish Setters And Other Dog Breeds With Hereditary Retinal Degeneration, Gustavo D. Aguirre, V Baldwin, K M. Weeks, Gregory M. Acland, Kunal Ray

Gustavo D. Aguirre, VMD, PhD

Rod-cone dysplasia 1 (rcd1) in Irish setters is caused by a nonsense mutation in the cGMP phosphodiesterase β-subunit gene (PDE6B). We examined the frequency of the mutant allele in the Irish setter population and determined if the defect is present in dogs of other breeds which are affected with other inherited photoreceptor diseases. Between 1994 and 1997, samples were obtained from 436 clinically normal Irish setters, a red wolf, and dogs from 23 different breeds. The mutation in codon 807 of PDE6B was detected in genomic DNA by heteroduplex analysis, allele-specific PCR, or restriction enzyme digestion. …