Evidence map›Paper›PMID 29122605›Full record

ArticleExperimental eye research2018

Phenotypic characterization of P23H and S334ter rhodopsin transgenic rat models of inherited retinal degeneration.

Matthew M LaVail, Shimpei Nishikawa, Roy H Steinberg, Muna I Naash, Jacque L Duncan, Nikolaus Trautmann, Michael T Matthes, Douglas Yasumura, Cathy Lau-Villacorta, Jeannie Chen and 3 more

Open access · greenAbstract read
In one paragraph

Article in Experimental eye research, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 58 papers.

0numbers the graph read from it
0cells of the map it votes in
58citing papers in PubMed
3.9field-weighted citation impact, top 5% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

58 citing papers in PubMed, 84 citations in OpenAlex.

  1. Article
  2. Article
  3. Article
  4. AAV NRF2 gene therapy preserves retinal structure and function in rodent models of oxidative damage.Molecular therapy : the journal of the American Society of Gene Therapy · 2026
    Article
  5. Article
  6. Article
  7. Article
  8. Early Rod Dysfunction Influences Cone Development in a Rhodopsin P23H Mouse Model of Retinitis Pigmentosa.Pathophysiology : the official journal of the International Society for Pathophysiology · 2026
    Article
  9. Article
  10. Review
  11. Review
  12. Animal models for the evaluation of retinal stem cell therapies.Progress in retinal and eye research · 2025
    Review
  13. Article
  14. RPE-specific MCT2 expression promotes cone survival in models of retinitis pigmentosa.Proceedings of the National Academy of Sciences of the United States of America · 2025
    Article
  15. Article
  16. Review
  17. Article
  18. Contribution of intraflagellar transport to compartmentalization and maintenance of the photoreceptor cell.Proceedings of the National Academy of Sciences of the United States of America · 2024
    Article
  19. Review
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

13 authors at 3 institutions in 1 country.

Matthew M LaVailBeckman Vision Center, University of California, San Francisco, San Francisco, CA 94143-0730, USA. Electronic address: mmlv@sonic.net.
Shimpei NishikawaBeckman Vision Center, University of California, San Francisco, San Francisco, CA 94143-0730, USA. Electronic address: shimpeinishikawa@yahoo.co.jp.
Roy H SteinbergBeckman Vision Center, University of California, San Francisco, San Francisco, CA 94143-0730, USA.
Muna I NaashDepartment of Biomedical Engineering, University of Houston, 3517 Cullen Blvd., Room 2011, Houston, TX 77204-5060, USA. Electronic address: mnaash@central.uh.edu.
Jacque L DuncanBeckman Vision Center, University of California, San Francisco, San Francisco, CA 94143-0730, USA. Electronic address: jacque.duncan@ucsf.edu.
Nikolaus TrautmannBeckman Vision Center, University of California, San Francisco, San Francisco, CA 94143-0730, USA. Electronic address: nick.trautmann@web.de.
Michael T MatthesBeckman Vision Center, University of California, San Francisco, San Francisco, CA 94143-0730, USA. Electronic address: michael.matthes@sbcglobal.net.
Douglas YasumuraBeckman Vision Center, University of California, San Francisco, San Francisco, CA 94143-0730, USA.
Cathy Lau-VillacortaBeckman Vision Center, University of California, San Francisco, San Francisco, CA 94143-0730, USA. Electronic address: clv95@yahoo.com.
Jeannie ChenZilka Neurogenetic Institute, USC Keck School of Medicine, Los Angeles, CA 90089-2821, USA. Electronic address: jeannie@usc.edu.
Ward M PetersonBeckman Vision Center, University of California, San Francisco, San Francisco, CA 94143-0730, USA. Electronic address: wardmpeterson@gmail.com.
Haidong YangBeckman Vision Center, University of California, San Francisco, San Francisco, CA 94143-0730, USA. Electronic address: yang.harvey@gmail.com.
John G FlannerySchool of Optometry, UC Berkeley, Berkeley, CA 94720-2020, USA. Electronic address: flannery@berkeley.edu.
University of California, San Francisco · USUniversity of California, Berkeley · USUniversity of Houston · US

Funding

Rapid-Prototyping and Design CoreP30EY002162 · NEI · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI LAMBA, DEEPAK ASHOK · 1985 to 2024
$16.8M
Phototransduction in health and diseaseR01EY012155 · NEI · UNIVERSITY OF SOUTHERN CALIFORNIA · PI Jeannie Chen · 1998 to 2026
$11.8M
Supplement for: Mechanism of Photoreceptor Cell Degeneration in Animal Models of Retinal DiseasesR01EY010609 · NEI · UNIVERSITY OF OKLAHOMA HLTH SCIENCES CTR · PI MUAYYAD R AL-UBAIDI, Muna I. Naash · 1995 to 2026
$11.0M
CELLULAR MECHANISMS OF INHERITED RETINAL DEGENERATIONR01EY001919 · NEI · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI LAVAIL, MATTHEW M · 1985 to 2014
$8.3M
Compacted DNA Nanoparticles for Ocular TherapyR01EY018656 · NEI · UNIVERSITY OF OKLAHOMA HLTH SCIENCES CTR · PI AL-UBAIDI, MUAYYAD R, NAASH, MUNA I. · 2008 to 2021
$5.6M
RODENT MODELS OF RETINAL DEGENERATIONR01EY006842 · NEI · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI LAVAIL, MATTHEW M · 1992 to 2014
$3.6M
Analyses of retinal circuits after rod rescue in a mouse model of human blindnessR01EY027193 · NEI · UNIVERSITY OF SOUTHERN CALIFORNIA · PI CHEN, JEANNIE, FIELD, GREGORY DARIN · 2016 to 2020
$2.7M
Neuronal networks for control of eye movementZ01EY000415 · NEI · NATIONAL EYE INSTITUTE · PI HIKOSAKA, OKIHIDE · 2003 to 2008
$2.3M
CALCIUM HOMEOSTASIS IN MAMMALIAN ROD AND CONE PHOTORECEPTORSR01EY027387 · NEI · WASHINGTON UNIVERSITY · PI CHEN, JEANNIE, KEFALOV, VLADIMIR JIVKOV · 2017 to 2020
$1.4M
Role of retbindin in retinal homeostasis and diseasesR01EY026499 · NEI · UNIVERSITY OF HOUSTON · PI AL-UBAIDI, MUAYYAD R, NAASH, MUNA I. · 2017 to 2019
$1.1M
THERAPY FOR DOMINANTLY INHERITED RETINAL DEGENERATIONSK08EY000415 · NEI · UNIVERSITY OF CALIFORNIA SAN FRANCISCO · PI DUNCAN, JACQUE L · 2000 to 2004
$684k
Mechanism of photoreceptor cell degeneration in animal models of retinal diseasesR56EY010609 · NEI · UNIVERSITY OF OKLAHOMA HLTH SCIENCES CTR · PI NAASH, MUNA I. · 2008 to 2008
$479k
Intramural NIH HHS Z01 EY000415NEI NIH HHS F32 EY006842NEI NIH HHS K08 EY000415NEI NIH HHS P30 EY002162NEI NIH HHS R01 EY001919NEI NIH HHS R01 EY006842NEI NIH HHS R01 EY010609NEI NIH HHS R01 EY012155NEI NIH HHS R01 EY018656NEI NIH HHS R01 EY026499NEI NIH HHS R01 EY027193NEI NIH HHS R01 EY027387NEI NIH HHS R56 EY010609
6 · The paper itself

Abstract

We produced 8 lines of transgenic (Tg) rats expressing one of two different rhodopsin mutations in albino Sprague-Dawley (SD) rats. Three lines were generated with a proline to histidine substitution at codon 23 (P23H), the most common autosomal dominant form of retinitis pigmentosa in the United States. Five lines were generated with a termination codon at position 334 (S334ter), resulting in a C-terminal truncated opsin protein lacking the last 15 amino acid residues and containing all of the phosphorylation sites involved in rhodopsin deactivation, as well as the terminal QVAPA residues important for rhodopsin deactivation and trafficking. The rates of photoreceptor (PR) degeneration in these models vary in proportion to the ratio of mutant to wild-type rhodopsin. The models have been widely studied, but many aspects of their phenotypes have not been described. Here we present a comprehensive study of the 8 Tg lines, including the time course of PR degeneration from the onset to one year of age, retinal structure by light and electron microscopy (EM), hemispheric asymmetry and gradients of rod and cone degeneration, rhodopsin content, gene dosage effect, rapid activation and invasion of the outer retina by presumptive microglia, rod outer segment disc shedding and phagocytosis by the retinal pigmented epithelium (RPE), and retinal function by the electroretinogram (ERG). The biphasic nature of PR cell death was noted, as was the lack of an injury-induced protective response in the rat models. EM analysis revealed the accumulation of submicron vesicular structures in the interphotoreceptor space during the peak period of PR outer segment degeneration in the S334ter lines. This is likely due to the elimination of the trafficking consensus domain as seen before as with other rhodopsin mutants lacking the C-terminal QVAPA. The 8 rhodopsin Tg lines have been, and will continue to be, extremely useful models for the experimental study of inherited retinal degenerations.

Indexed as

Disease Models, AnimalPoint MutationAnimalsElectroretinographyMicroscopyMicroscopy, ElectronPhenotypePhotoreceptor Cells, VertebratePolymerase Chain ReactionRatsRats, Sprague-DawleyRats, TransgenicRetinaRetinal DegenerationRhodopsinRhodopsinAnimal modelP23HRatRetinitis pigmentosaRhodopsinS334ter

Identifiers

PMID29122605
PMCPMC5811379
OpenAlexW2767585027

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.