Open Access. Powered by Scholars. Published by Universities.®

Medicine and Health Sciences Commons™

Open Access. Powered by Scholars. Published by Universities.®

Veterinary Medicine

Selected Works

2001

Articles 1 - 7 of 7

Full-Text Articles in Medicine and Health Sciences

Concentric Retinitis Pigmentosa: Clinicopathologic Correlations, Ann Milam, Elaine De Castro, Julie Smith, Wai-Xing Tang, Sinoj John, Michael Gorin, Edwin Stone, Gustavo Aguirre, Samuel Jacobson Sep 2001

Concentric Retinitis Pigmentosa: Clinicopathologic Correlations, Ann Milam, Elaine De Castro, Julie Smith, Wai-Xing Tang, Sinoj John, Michael Gorin, Edwin Stone, Gustavo Aguirre, Samuel Jacobson

Gustavo D. Aguirre, VMD, PhD


Progressive concentric (centripetal) loss of vision is one pattern of visual field loss in retinitis pigmentosa. This study provides the first clinicopathologic correlations for this form of retinitis pigmentosa. A family with autosomal dominant concentric retinitis pigmentosa was examined clinically and with visual function tests. A post-mortem eye of an affected 94 year old family member was processed for histopathology and immunocytochemistry with retinal cell specific antibodies. Unrelated simplex/multiplex patients with concentric retinitis pigmentosa were also examined. Affected family members of the eye donor and patients from the other families had prominent peripheral pigmentary retinopathy with more normal appearing central …

Go to article

Fine Mapping Of Canine Xlpra Establishes Homology Of The Human And Canine Rp3 Intervals, Qi Zhang, Gregory M. Acland, Barbara Zangerl, J. L. Johnson, Zuohua Mao, Caroline Zeiss, Elaine A. Ostrander, Gustavo D. Aguirre Sep 2001

Fine Mapping Of Canine Xlpra Establishes Homology Of The Human And Canine Rp3 Intervals, Qi Zhang, Gregory M. Acland, Barbara Zangerl, J. L. Johnson, Zuohua Mao, Caroline Zeiss, Elaine A. Ostrander, Gustavo D. Aguirre

Gustavo D. Aguirre, VMD, PhD

Purpose: Canine X-linked progressive retinal atrophy (XLPRA) is a hereditary, progressive retinal degeneration that has been mapped previously to the canine X chromosome in a region flanked by the dystrophin (DMD) and tissue inhibitor of metalloproteinase 1 (TIMP1) genes, and is tightly linked to the gene RPGR. The comparable region of the human X chromosome includes the disease locus for RP3, an X-linked form of retinitis pigmentosa, although the current canine disease interval is much larger.
Methods: To refine the map of the canine XLPRA disease interval, 11 X-linked markers were mapped, both meiotically, in two extensive …

Go to article

Cloning Of Canine Γ-Tubulin (Tubg1) Cdna And Mapping To Cfa9, Duska J. Sidjanin, F Xue, J Mcelwee, Jennifer L. Johnson, C Holmgren, Cathryn S. Mellersh, Elaine A. Ostrander, Gregory M. Acland, Gustavo D. Aguirre Sep 2001

Cloning Of Canine Γ-Tubulin (Tubg1) Cdna And Mapping To Cfa9, Duska J. Sidjanin, F Xue, J Mcelwee, Jennifer L. Johnson, C Holmgren, Cathryn S. Mellersh, Elaine A. Ostrander, Gregory M. Acland, Gustavo D. Aguirre

Gustavo D. Aguirre, VMD, PhD

No abstract provided.

Go to article

Gene Therapy Restores Vision In A Canine Model Of Childhood Blindness, Gregory M. Acland, Gustavo D. Aguirre, Jharna Ray, Qi Zhang, Tomas S. Aleman, Artur V. Cideciyan, Susan E. Pearce-Kelling, Vibha Anand, Yong Zeng, Albert M. Maguire, Samuel G. Jacobson, William W. Hauswirth, Jean Bennett Apr 2001

Gene Therapy Restores Vision In A Canine Model Of Childhood Blindness, Gregory M. Acland, Gustavo D. Aguirre, Jharna Ray, Qi Zhang, Tomas S. Aleman, Artur V. Cideciyan, Susan E. Pearce-Kelling, Vibha Anand, Yong Zeng, Albert M. Maguire, Samuel G. Jacobson, William W. Hauswirth, Jean Bennett

Gustavo D. Aguirre, VMD, PhD

The relationship between the neurosensory photoreceptors and the adjacent retinal pigment epithelium (RPE) controls not only normal retinal function, but also the pathogenesis of hereditary retinal degenerations. The molecular bases for both primary photoreceptor1 and RPE diseases2, 3, 4 that cause blindness have been identified. Gene therapy has been used successfully to slow degeneration in rodent models of primary photoreceptor diseases5, 6, but efficacy of gene therapy directed at photoreceptors and RPE in a large-animal model of human disease has not been reported. Here we study one of the most clinically severe retinal degenerations, Leber congenital amaurosis (LCA). LCA causes …

Go to article

Evaluation Of Retinal Photoreceptors And Pigment Epithelium In A Female Carrier Of Choroideremia, Nasreen Syed, Julie E. Smith, Sinoj K. John, Miguel C. Seabra, Gustavo D. Aguirre, Ann H. Milam Mar 2001

Evaluation Of Retinal Photoreceptors And Pigment Epithelium In A Female Carrier Of Choroideremia, Nasreen Syed, Julie E. Smith, Sinoj K. John, Miguel C. Seabra, Gustavo D. Aguirre, Ann H. Milam

Gustavo D. Aguirre, VMD, PhD

Purpose: To clarify the pathogenesis of choroideremia.
Study Design: Human tissue study.
Tissues: Eyes of an 88-year-old symptomatic female carrier of choroideremia (CHM) and six normal, age-matched donors.
Methods: The eyes were processed for histopathologic examination, including immunocytochemistry with an antibody against the CHM gene product, REP-1, and retinal cell-specific markers.
Results: The CHM carrier retina showed patchy degeneration, but the photoreceptor and retinal pigment epithelium (RPE) loss appeared to be independent. The choriocapillaris was normal except where retinal areas were severely degenerate. The CHM gene product, REP-1, was localized to the cytoplasm of rods but not cones.
Conclusions: It …

Go to article

Abnormal Expression Of P27kip1 Protein In Levator Ani Muscle Of Aging Women With Pelvic Floor Disorders – A Relationship To The Cellular Differentiation And Degeneration, Antonin Bukovsky, Pleas Copas, Michael R. Caudle, Maria Cekanova Ms, Rndr, Phd, Tamara Dassanayake, Bridgett Asbury, Stuart E. Van Meter, Robert F. Elder, Jeffrey B. Brown, Stephanie B. Cross Jan 2001

Abnormal Expression Of P27kip1 Protein In Levator Ani Muscle Of Aging Women With Pelvic Floor Disorders – A Relationship To The Cellular Differentiation And Degeneration, Antonin Bukovsky, Pleas Copas, Michael R. Caudle, Maria Cekanova Ms, Rndr, Phd, Tamara Dassanayake, Bridgett Asbury, Stuart E. Van Meter, Robert F. Elder, Jeffrey B. Brown, Stephanie B. Cross

Maria Cekanova MS, RNDr, PhD

Background Pelvic floor disorders affect almost 50% of aging women. An important role in the pelvic floor support belongs to the levator ani muscle. The p27/kip1 (p27) protein, multifunctional cyclin-dependent kinase inhibitor, shows changing expression in differentiating skeletal muscle cells during development, and relatively high levels of p27 RNA were detected in the normal human skeletal muscles.

Methods Biopsy samples of levator ani muscle were obtained from 22 symptomatic patients with stress urinary incontinence, pelvic organ prolapse, and overlaps (age range 38–74), and nine asymptomatic women (age 31–49). Cryostat sections were investigated for p27 protein expression and type I (slow …

Go to article

Adenoviral Vector-Mediated Ss-Glucuronidase Cdna Transfer To Treat Mps Vii Rpe In Vitro, Maria E. Verdugo, Virginia Scarpino, Phillipe Moullier, M Haskins, Gustavo D. Aguirre, Jharna Ray Dec 2000

Adenoviral Vector-Mediated Ss-Glucuronidase Cdna Transfer To Treat Mps Vii Rpe In Vitro, Maria E. Verdugo, Virginia Scarpino, Phillipe Moullier, M Haskins, Gustavo D. Aguirre, Jharna Ray

Gustavo D. Aguirre, VMD, PhD

Purpose. To develop an effective therapy for treating glycosaminoglycan (GAG) storage in mucopolysaccharidosis VII (MPS VII) retinal pigment epithelium (RPE) in vitro using adenoviral vector mediated human ß-glucuronidase cDNA (Ad-GUSB) transfer. Methods. Ad-GUSB was used to infect RPE at confluency. The transduction condition was optimized varying time of infection and number of infectious particles. The ß-glucuronidase (GUSB) activity was measured in transduced cells and media using a fluorogenic substrate. The GAG profiles were examined by metabolically labeling RPE with 35 Na 2 SO 4. Results. Transduced RPE, irrespective of species or disease status, expressed a high level of ß-glucuronidase. The …

Go to article