Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

A peripheral subpopulation of retinal pigment epithelium resists oxidative damage through SERPINE3-mediated Caspase-1 inhibition.

The Journal of clinical investigation·2026
Same author

Structural variant discovery and diagnostic impact in rare diseases from short-read and long-read sequencing.

medRxiv : the preprint server for health sciences·2026
Same author

Mucopolysaccharidosis type I with retinal degeneration and absence of corneal involvement in the eighth decade of life.

Ophthalmic genetics·2026
Same author

Low temperature selective catalytic oxidation of ammonia reaction (NH<sub>3</sub>-SCO): Introducing Co, or Zr to improve comprehensive performance of the Ag/Al<sub>2</sub>O<sub>3</sub> catalyst.

Journal of hazardous materials·2026
Same author

Association of Autosomal Dominant Snowflake Vitreoretinal Degeneration with Retinoschisis.

Ophthalmology science·2026
Same author

High-Density Lipoprotein Nanoparticles Delivering Liver X Receptor Agonist for the Treatment of Age-Related Macular Degeneration.

Molecular pharmaceutics·2026

Related Experiment Video

Updated: Aug 31, 2025

Assessing Early Stage Open-Angle Glaucoma in Patients by Isolated-Check Visual Evoked Potential
07:11

Assessing Early Stage Open-Angle Glaucoma in Patients by Isolated-Check Visual Evoked Potential

Published on: May 25, 2020

6.5K

Baseline Microperimetry and OCT in the RUSH2A Study: Structure-Function Association and Correlation With Disease

Eleonora M Lad1, Jacque L Duncan2, Wendi Liang3

  • 1From the Department of Ophthalmology, Duke University Medical Center Durham, North Carolina, USA.

American Journal of Ophthalmology
|August 25, 2022
PubMed
Summary
This summary is machine-generated.

Longer disease duration in USH2A-related retinal degeneration correlates with worsening vision and retinal structure. Microperimetry and OCT metrics show significant associations, aiding disease progression insights.

More Related Videos

In vivo Structural Assessments of Ocular Disease in Rodent Models using Optical Coherence Tomography
07:44

In vivo Structural Assessments of Ocular Disease in Rodent Models using Optical Coherence Tomography

Published on: July 24, 2020

3.0K
Ultrahigh Resolution Mouse Optical Coherence Tomography to Aid Intraocular Injection in Retinal Gene Therapy Research
10:10

Ultrahigh Resolution Mouse Optical Coherence Tomography to Aid Intraocular Injection in Retinal Gene Therapy Research

Published on: November 2, 2018

9.4K

Related Experiment Videos

Last Updated: Aug 31, 2025

Assessing Early Stage Open-Angle Glaucoma in Patients by Isolated-Check Visual Evoked Potential
07:11

Assessing Early Stage Open-Angle Glaucoma in Patients by Isolated-Check Visual Evoked Potential

Published on: May 25, 2020

6.5K
In vivo Structural Assessments of Ocular Disease in Rodent Models using Optical Coherence Tomography
07:44

In vivo Structural Assessments of Ocular Disease in Rodent Models using Optical Coherence Tomography

Published on: July 24, 2020

3.0K
Ultrahigh Resolution Mouse Optical Coherence Tomography to Aid Intraocular Injection in Retinal Gene Therapy Research
10:10

Ultrahigh Resolution Mouse Optical Coherence Tomography to Aid Intraocular Injection in Retinal Gene Therapy Research

Published on: November 2, 2018

9.4K

Area of Science:

  • Ophthalmology
  • Genetics
  • Retinal Diseases

Background:

  • USH2A gene variants cause Usher syndrome type 2 (USH2) and nonsyndromic autosomal recessive retinitis pigmentosa (ARRP).
  • Understanding the natural history of USH2A-related retinal degeneration is crucial for developing effective treatments.

Purpose of the Study:

  • To investigate baseline mesopic microperimetry (MP) and spectral domain optical coherence tomography (OCT) in the USH2A-related Retinal Degeneration (RUSH2A) study.
  • To establish correlations between retinal structure and function in patients with USH2A variants.

Main Methods:

  • A natural history study conducted at 16 clinical sites across Europe and North America.
  • Participants included 80 individuals with USH2 and 47 with ARRP associated with USH2A variants.
  • General linear models and Spearman correlation coefficients were used to analyze MP (mean sensitivity) and OCT (intact ellipsoid zone area, central subfield thickness) data in relation to disease duration, best corrected visual acuity (BCVA).

Main Results:

  • OCT and MP data were obtained from 127 and 93 participants, respectively.
  • No significant differences in EZ area, CST, or mean sensitivity were observed between USH2 and ARRP groups.
  • Longer disease duration was associated with smaller EZ area and lower mean sensitivity (P < 0.001 and P = 0.01, respectively).
  • Greater mean sensitivity correlated with better BCVA, larger EZ area, and larger CST (r > 0.3, P < 0.01).
  • Better BCVA and larger CST were associated with larger EZ area (r > 0.6, P < 0.001).

Conclusions:

  • Longer disease duration correlates with more severe retinal structure and function abnormalities in USH2A-related retinal degeneration.
  • Significant associations exist between microperimetry and optical coherence tomography metrics.
  • Monitoring these structure-function relationships is vital for understanding disease mechanisms and progression.