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Full-Text Articles in Medicine and Health Sciences
Raav2/5 Gene-Targeting To Rods: Dose-Dependent Efficiency And Complications Associated With Different Promoters, William Beltran, Sanford L. Boye, Shannon E. Boye, Vince A. Chiodo, Alfred S. Lewin, William W. Hauswirth, Gustavo D. Aguirre
Raav2/5 Gene-Targeting To Rods: Dose-Dependent Efficiency And Complications Associated With Different Promoters, William Beltran, Sanford L. Boye, Shannon E. Boye, Vince A. Chiodo, Alfred S. Lewin, William W. Hauswirth, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
A prerequisite for using corrective gene therapy to treat humans with inherited retinal degenerative diseases that primarily affect rods is to develop viral vectors that target specifically this population of photoreceptors. The delivery of a viral vector with photoreceptor tropism coupled with a rod-specific promoter is likely to be the safest and most efficient approach to target expression of the therapeutic gene to rods. Three promoters that included a fragment of the proximal mouse opsin promoter (mOP), the human G-protein-coupled receptor protein kinase 1 promoter (hGRK1), or the cytomegalovirus immediate early enhancer combined with the chicken β actin proximal promoter …
Photoreceptor Dysplasia: An Inherited Progressive Retinal Atrophy Of Miniature Schnauzer Dogs, Charles J. Parshall, Milton Wyman, Susan Nitroy, Gregory M. Acland, Gustavo D. Aguirre
Photoreceptor Dysplasia: An Inherited Progressive Retinal Atrophy Of Miniature Schnauzer Dogs, Charles J. Parshall, Milton Wyman, Susan Nitroy, Gregory M. Acland, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
A progressive retinal atrophy (PRA) affecting Miniature Schnauzer dogs is reported. Of the 287 individuals (148 female, 139 male) comprising the study population, 66 (23 percent) were affected (33 female, 33 male) and 221 animals (115 female, 106 male) were phenotypically normal. There was no sex predilection for the disease. Results of histologic and electroretinographic studies indicate that the disease is a new and different type of PRA, characterized by unique morphologic and functional deficits during rod and cone development. Accordingly, the disease has been termed photoreceptor dysplasia. Clinically, and particularly ophthalmoscopically, diagnosis is only practicable in very late stages …
Posterior Lenticonus In The Dog, Gustavo D. Aguirre, Stephen I. Bistner
Posterior Lenticonus In The Dog, Gustavo D. Aguirre, Stephen I. Bistner
Gustavo D. Aguirre, VMD, PhD
Posterior lenticonus is a congenital defect of the posterior lenticular surface. The posterior cortical and capsular regions of the lens have a circumscribed conelike or globular protrusion of variable size. Opacities may be present in the region of the conus. The defect has been reported in man, rabbits, calves and mice. This report documents 2 cases in unrelated dogs. The possible mechanism for the formation of this defect is discussed.
Posterior Segment Approach For Subretinal Transplantation Or Injection In The Canine Model, Maria E. Verdugo, Julie Alling, Eliot Lazar, Manuel Del Cerro, Jharna Ray, Gustavo D. Aguirre
Posterior Segment Approach For Subretinal Transplantation Or Injection In The Canine Model, Maria E. Verdugo, Julie Alling, Eliot Lazar, Manuel Del Cerro, Jharna Ray, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
A posterior segment approach for cell transplantation or injection into the subretinal space of the dog has been developed. Controlled penetration to the subretinal space was achieved using a 29-gauge injection cannula, either blunted or with a 30° sharpened bevel, and partially ensheathed with moveable plastic tubing. Depending on the injection volume used, the retina detached, and the fluid was reabsorbed within 1–3 weeks, although for smaller volumes the retina reattached within a matter of days. The optimal injection volume used was between 100 and 150 μl, or two injections of 55 μl each. By ophthalmoscopy following the surgery, it …
Assessment Of Canine Best1 Variations Identifies New Mutations And Establishes An Independent Bestrophinopathy Model (Cmr3), Barbara Zangerl, Kaisa Wickström, Julianna Slavik, Sarah J. Lindauer, Saija Ahonen, Claude Schelling, Hannes Lohi, Karina E. Guziewicz, Gustavo D. Aguirre
Assessment Of Canine Best1 Variations Identifies New Mutations And Establishes An Independent Bestrophinopathy Model (Cmr3), Barbara Zangerl, Kaisa Wickström, Julianna Slavik, Sarah J. Lindauer, Saija Ahonen, Claude Schelling, Hannes Lohi, Karina E. Guziewicz, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
Purpose: Mutations in bestrophin 1 (BEST1) are associated with a group of retinal disorders known as bestrophinopathies in man and canine multifocal retinopathies (cmr) in the dog. To date, the dog is the only large animal model suitable for the complex characterization and in-depth studies of Best-related disorders. In the first report of cmr, the disease was described in a group of mastiff-related breeds (cmr1) and the Coton de Tulear (cmr2). Additional breeds, e.g., the Lapponian herder (LH) and others, subsequently were recognized with similar phenotypes, but linked loci are unknown. …
A Kutya Mint Modell Az Örökletes Retinadegenerációk Kutatásában / The Dog As A Model In Research Into Inherited Retinal Degenerations, Ágnes I. Berta, Ágoston Szél, Gustavo D. Aguirre
A Kutya Mint Modell Az Örökletes Retinadegenerációk Kutatásában / The Dog As A Model In Research Into Inherited Retinal Degenerations, Ágnes I. Berta, Ágoston Szél, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
Összefoglalás: A kutya mint modellállat csak az elmúlt 15 évben került az érdeklődés középpontjába. A kutya retinája különösen érdekes, mivel számos örökletes retinadegeneráció szoros homológiát mutat az ember hasonló betegségeivel. Röviden összefoglaljuk a kutya retinájának sajátosságait és azokat az alapkutatásban használt módszereket, melyek segítségével a retina degenerációja leírható kutyában. A kutya örökletes retinadegenerációi közül a szemészek számára a legérdekesebb csoport a progresszív retinalis atrophia (PRA), mivel genetikai, patológiai és klinikai szempontból is nagyon hasonló a retinitis pigmentosa különböző típusaihoz. A PRA csoport tagjai élő modellrendszerek, melyek alkalmazhatók az örökletes retinadegenerációk alap- és klinikai kutatásában egyaránt. Ezekben a súlyos és mindenekelőtt …
Application Of A New Subretinal Injection Device In The Dog, András M. Komáromy, Signe E. Varner, Eugene De Juan, Gregory M. Acland, Gustavo D. Aguirre
Application Of A New Subretinal Injection Device In The Dog, András M. Komáromy, Signe E. Varner, Eugene De Juan, Gregory M. Acland, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
The use of a new subretinal injection device (RetinaJect™ Subretinal Cannula, SurModics, Inc., Eden Prairie, MN) to access the subretinal space in the canine model was evaluated. Subretinal injections were performed in 33 mongrel dogs between 2 and 52 months of age (median = 9 months). In 5 normal dogs the injection of 150 μl saline or India ink occurred by using a conventional subretinal injection device (CSID) with a 30-gauge anterior chamber irrigating cannula. The sclera had to be surgically exposed and penetrated before the subretinal injection with the CSID could occur. After removing the CSID, the conjunctiva over …
Intravitreal Injection Of Ciliary Neurotrophic Factor (Cntf) Causes Peripheral Remodeling And Does Not Prevent Photoreceptor Loss In Canine Rpgr Mutant Retina, William Beltran, Rong Wen, Gregory M. Acland, Gustavo D. Aguirre
Intravitreal Injection Of Ciliary Neurotrophic Factor (Cntf) Causes Peripheral Remodeling And Does Not Prevent Photoreceptor Loss In Canine Rpgr Mutant Retina, William Beltran, Rong Wen, Gregory M. Acland, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
Ciliary neurotrophic factor (CNTF) rescues photoreceptors in several animal models of retinal degeneration and is currently being evaluated as a potential treatment for retinitis pigmentosa in humans. This study was conducted to test whether CNTF prevents photoreceptor cell loss in XLPRA2, an early onset canine model of X-linked retinitis pigmentosa caused by a frameshift mutation in RPGR exon ORF15. Four different treatment regimens of CNTF were tested in XLPRA2 dogs. Under anesthesia, the animals received at different ages an intravitreal injection of 12 μg of CNTF in the left eye. The right eye served as a control and was injected …
A Meiotic Linkage Map Of The Silver Fox, Aligned And Compared To The Canine Genome, Anna V. Kukekova, Lyudmila N. Trut, Irina N. Oskina, Jennifer L. Johnson, Svetlana V. Temnykh, Anastasiya V. Kharlamova, Darya V. Shepeleva, Rimma G. Gulievich, Svetlana G. Shikhevich, Alexander S. Graphodatsky, Gustavo D. Aguirre, Gregory M. Acland
A Meiotic Linkage Map Of The Silver Fox, Aligned And Compared To The Canine Genome, Anna V. Kukekova, Lyudmila N. Trut, Irina N. Oskina, Jennifer L. Johnson, Svetlana V. Temnykh, Anastasiya V. Kharlamova, Darya V. Shepeleva, Rimma G. Gulievich, Svetlana G. Shikhevich, Alexander S. Graphodatsky, Gustavo D. Aguirre, Gregory M. Acland
Gustavo D. Aguirre, VMD, PhD
A meiotic linkage map is essential for mapping traits of interest and is often the first step toward understanding a cryptic genome. Specific strains of silver fox (a variant of the red fox, Vulpes vulpes), which segregate behavioral and morphological phenotypes, create a need for such a map. One such strain, selected for docility, exhibits friendly dog-like responses to humans, in contrast to another strain selected for aggression. Development of a fox map is facilitated by the known cytogenetic homologies between the dog and fox, and by the availability of high resolution canine genome maps and sequence data. Furthermore, the …
Radiation Hybrid Map, Physical Map, And Low-Pass Genomic Sequence Of The Canine Prcd Region On Cfa9 And Comparative Mapping With The Syntenic Region On Human Chromosome 17 ☆, Duska Sidjanin, B Miller, J. W. Kijas, J Mcelwee, Jarek Pillardy, J Malek, G Pai, Tamara Feldblyum, C Fraser, Gregory Acland, Gustavo Aguirre
Radiation Hybrid Map, Physical Map, And Low-Pass Genomic Sequence Of The Canine Prcd Region On Cfa9 And Comparative Mapping With The Syntenic Region On Human Chromosome 17 ☆, Duska Sidjanin, B Miller, J. W. Kijas, J Mcelwee, Jarek Pillardy, J Malek, G Pai, Tamara Feldblyum, C Fraser, Gregory Acland, Gustavo Aguirre
Gustavo D. Aguirre, VMD, PhD
Comparative Analysis, Gene Organization And Expression Of Canine Tcte1l, Kui Li, Qi Zhang, Jennifer L. Johnson, Gustavo D. Aguirre
Comparative Analysis, Gene Organization And Expression Of Canine Tcte1l, Kui Li, Qi Zhang, Jennifer L. Johnson, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
Molecular Cloning, Characterization And Expression Of A Novel Retinal Clusterin-Like Protein Cdna, Qi Zhang, Kunal Ray, Gregory M. Acland, Jill M. Czarnecki, Gustavo D. Aguirre
Molecular Cloning, Characterization And Expression Of A Novel Retinal Clusterin-Like Protein Cdna, Qi Zhang, Kunal Ray, Gregory M. Acland, Jill M. Czarnecki, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
Evaluation Of Cgmp-Phosphodiesterase (Pde) Subunits For Causal Association With Rod–Cone Dysplasia 2 (Rcd2), A Canine Model Of Abnormal Retinal Cgmp Metabolism, Weiquan Wang, Gregory Acland, Kunal Ray, Gustavo Aguirre
Evaluation Of Cgmp-Phosphodiesterase (Pde) Subunits For Causal Association With Rod–Cone Dysplasia 2 (Rcd2), A Canine Model Of Abnormal Retinal Cgmp Metabolism, Weiquan Wang, Gregory Acland, Kunal Ray, Gustavo Aguirre
Gustavo D. Aguirre, VMD, PhD
Timp-1 Expression Is Increased In X-Linked Progressive Retinal Atrophy Despite Its Exclusion As A Candidate Gene, Caroline Zeiss, Gregory M. Acland, Gustavo D. Aguirre, Kunal Ray
Timp-1 Expression Is Increased In X-Linked Progressive Retinal Atrophy Despite Its Exclusion As A Candidate Gene, Caroline Zeiss, Gregory M. Acland, Gustavo D. Aguirre, Kunal Ray
Gustavo D. Aguirre, VMD, PhD
Canine Rod Photoreceptor Cgmp-Gated Channel Protein Α-Subunit: Studies On The Expression Of The Gene And Characterization Of The Cdna, Qi Zhang, Sue Pearce-Kelling, Gregory Acland, Gustavo Aguirre, Kunal Ray
Canine Rod Photoreceptor Cgmp-Gated Channel Protein Α-Subunit: Studies On The Expression Of The Gene And Characterization Of The Cdna, Qi Zhang, Sue Pearce-Kelling, Gregory Acland, Gustavo Aguirre, Kunal Ray
Gustavo D. Aguirre, VMD, PhD
Differential Expression Of Photoreceptor-Specific Proteins During Disease And Degeneration In The Progressive Rod-Cone Degeneration (Prcd) Retina, Kathryn Gropp, Jun Huang, Gustavo Aguirre
Differential Expression Of Photoreceptor-Specific Proteins During Disease And Degeneration In The Progressive Rod-Cone Degeneration (Prcd) Retina, Kathryn Gropp, Jun Huang, Gustavo Aguirre
Gustavo D. Aguirre, VMD, PhD
Canine Rod Transducin A-1: Cloning Of The Cdna And Evaluation Of The Gene As A Candidate For Progressive Retinal Atrophy, Kunal Ray, Victoria J. Baldwin, Caroline Zeiss, Gregory M. Acland, Gustavo D. Aguirre
Canine Rod Transducin A-1: Cloning Of The Cdna And Evaluation Of The Gene As A Candidate For Progressive Retinal Atrophy, Kunal Ray, Victoria J. Baldwin, Caroline Zeiss, Gregory M. Acland, Gustavo D. Aguirre
Gustavo D. Aguirre, VMD, PhD
Selective Absence Of Cone Outer Segment Β3-Transducin Immunoreactivity In Hereditary Cone Degeneration (Cd), Kathryn Gropp, A. Széll, Jun Huang, Gregory Acland, Debora Farber, Gustavo Aguirre
Selective Absence Of Cone Outer Segment Β3-Transducin Immunoreactivity In Hereditary Cone Degeneration (Cd), Kathryn Gropp, A. Széll, Jun Huang, Gregory Acland, Debora Farber, Gustavo Aguirre
Gustavo D. Aguirre, VMD, PhD
Decreased Opsin Mrna And Immunoreactivity In Progressive Rod-Cone Degeneration (Prcd): Cytochemical Studies Of Early Disease And Degeneration, Jun Huang, Marie-Françoise Chesselet, Gustavo Aguirre
Decreased Opsin Mrna And Immunoreactivity In Progressive Rod-Cone Degeneration (Prcd): Cytochemical Studies Of Early Disease And Degeneration, Jun Huang, Marie-Françoise Chesselet, Gustavo Aguirre
Gustavo D. Aguirre, VMD, PhD
Interphotoreceptor Retinoid-Binding Protein (Irbp) In Progressive Rod-Cone Degeneration (Prcd)—Biochemical, Immunocytochemical And Immunologic Studies, Gustavo Aguirre, Barbara Wiggert, Geetha Kutty, Kenneth Long, Lila Inouye, Igal Gery, Gerald Chader
Interphotoreceptor Retinoid-Binding Protein (Irbp) In Progressive Rod-Cone Degeneration (Prcd)—Biochemical, Immunocytochemical And Immunologic Studies, Gustavo Aguirre, Barbara Wiggert, Geetha Kutty, Kenneth Long, Lila Inouye, Igal Gery, Gerald Chader
Gustavo D. Aguirre, VMD, PhD
The Cone Matrix Sheath In The Normal And Diseased Retina: Cytochemical And Biochemical Studies Of Peanut Agglutinin-Binding Proteins In Cone And Rod-Cone Degeneration, Kenneth Long, Gustavo Aguirre
The Cone Matrix Sheath In The Normal And Diseased Retina: Cytochemical And Biochemical Studies Of Peanut Agglutinin-Binding Proteins In Cone And Rod-Cone Degeneration, Kenneth Long, Gustavo Aguirre
Gustavo D. Aguirre, VMD, PhD
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
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
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
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
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
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