New Insights Into Retinal Degenerative Diseases

1. Front Matter

   Pages i-xii

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2. Molecular Biology Studies in Human Retinal Degenerations

   1. Clinical Spectrum in Autosomal Dominant Stargardt’s Macular Dystrophy with a Mutation in ELOVL4 Gene

      • Yang Li, Linda A. Lam, Zhengya Yu, Zhenglin Yang, Paul Bither, Kang Zhang

      Pages 1-9

   2. X-Linked Retinitis Pigmentosa: Current Status

      • Debra K. Breuer, Maurizio Affer, Sten Andreasson, David G. Birch, Gerald A. Fishman, John R. Heckenlively et al.

      Pages 11-22

   3. Clinical Variabity of Patients Associated with RDH5 Gene Mutation

      • Yuko Wada, Toshiaki Abe, Miyuki Kawamura, Makoto Tamai

      Pages 23-28

   4. Japanese Patients with Fundus Albipunctatus Caused by RDH5 Gene Mutations

      • Makoto Nakamura, Yoshihiro Hotta, Yozo Miyake

      Pages 29-35

   5. Functional Analysis of AIPL1

      • Melanie M. Sohocki, Dayna L. Tirpak, Cheryl M. Craft, Stephen P. Daiger

      Pages 37-44

   6. Searching for Genotype-Phenotype Correlations in X-Linked Juvenile Retinoschisis

      • Kelaginamane T. Hiriyanna, Rita Singh-Parikshak, Eve L. Bingham, Jennifer A. Kemp, Radha Ayyagari, Beverly M. Yashar et al.

      Pages 45-53

   7. RP1 Mutation Analysis

      • Sara J. Bowne, Stephen P. Daiger, Kimberly A. Malone, Jin Zuo, Kyeongmi Cheon, David G. Birch et al.

      Pages 55-61

   8. The Molecular Basis of Achromatopsia

      • Susanne Kohl, Herbert Jägle, Eberhart Zrenner, Lindsay T. Sharpe, Bernd Wissinger

      Pages 63-70

   9. Characterisation of the CRX Gene; Identification of Alternatively Spliced 5’ Exons and 3’ Sequence

      • Matthew D. Hodges, Kevin Gregory-Evans, Cheryl Y. Gregory-Evans

      Pages 71-85

   10. Rhodopsin Mutations in Sectorial Retinitis Pigmentosa

       • S. Kaushal, D. A. R. Bessant, A. M. Payne, S. M. Downes, G. E. Holder, S. S. Bhattacharya et al.

       Pages 87-94

3. Treatment

   1. Chronic Administration of Phenyl N-TERT- Butylnitrone Protects the Retina Against Light Damage

      • Isabelle Ranchon, Jeremy White, Sherry Chen, Kathleen Alvarez, Yashige Kotake, Robert E. Anderson

      Pages 95-103

   2. Systems for Delivery of Vitamin A to the Retina in Retinitis Pigmentosa

      • Ekaterina M. Semenova, Alan Cooper, Malcolm W. Kennedy, Clive G. Wilson, Carolyn A. Converse

      Pages 105-110

   3. Timecourse of bFGF and CNTF Expression in Light-Induced Photoreceptor Degeneration in the Rat Retina

      • Natalie Walsh, Jonathan Stone

      Pages 111-118

   4. Role of Pigment Epithelium-Derived Factor (PEDF) in Photoreceptor Cell Protection

      • Wei Cao, Joyce Tombran-Tink, Rajesh Elias, Steven Sezate, James F. McGinnis

      Pages 119-126

   5. Characterization and Localization of Pigment Epithelium-Derived Factor Binding Sites in the Bovine Retina

      • Maria S. Aymerich, Alfredo Martinez, S. Patricia Becerra

      Pages 127-133

   6. Leukemia Inhibitory Factor Prevents Photoreceptor Cell Death in rd-/- Mice by Blocking Functional Differentiation

      • John D. Ash

      Pages 135-144

   7. Glycobiological Approach to Control of Retinal Degeneration

      • Fumiyuki Uehara, Norio Ohba, Masayuki Ozawa

      Pages 145-152

   8. A Multidisciplinary Approach to Investigating Optic Nerve Regeneration in the Goldfish

      • Malini Devadas, Toru Matsukawa, Zhongwu Liu, Kayo Sugitani, Kiyoshi Sugawara, Manabu Kaneda et al.

      Pages 153-161

   9. Intact-Sheet Fetal Retinal Transplants can Repair Degenerated Retinas

      • Magdalene J. Seiler, Robert B. Aramant

      Pages 163-173

Since 1984, we have organized satellite symposia on retinal degenerations that are held in conjunction with the biennial International Congress of Eye Research. The timing and location of our Retinal Degeneration Symposia have allowed scientists and clinicians from around the world to convene and present their exciting new findings. The symposia have been arranged to allow ample time for discussions and one-on-one interactions in a relaxed atmosphere, where international friendships and collaborations could be established. The IXth International Symposium on Retinal Degeneration was held on October 9-14, 2000 in Durango, Colorado and was attended by over 100 scientists from six continents. This book contains many of their presentations. Several events of note occurred at this meeting. First, thanks to the generous support of the Foundation Fighting Blindness, we were able to sponsor the travel of 11 young scientists from six countries. Most of them have contributed chapters to this volume. The response to the travel program was so overwhelming that we will make it regular feature of our meeting. This will allow other bright, young investigators to be introduced to the world experts who study retinal degenerations. Second, about 40% of the scientists who attended this meeting were there for the first time. We believe that this indicates a growing interest in retinal degeneration research and ensures that new talent will be attracted to this important area of investigation. The symposium received support from several organizations.

• DNA
• Invertebrate
• Regeneration
• Vertebrate
• gene therapy
• genes
• genetics
• molecular biology
• phenotype

• Dean A. McGee Eye Institute, University of Oklahoma Health Sciences Center, Oklahoma City, USA

  Robert E. Anderson

• Beckman Vision Center, University of California, San Francisco, San Francisco, USA

  Matthew M. LaVail

• Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, USA

  Joe G. Hollyfield

Policies and ethics