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 Experiment Videos

The pupil in dominant optic atrophy.

F D Bremner1, E A Tomlin, J Shallo-Hoffmann

  • 1National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK. fdbremner@doctors.org.uk

Investigative Ophthalmology & Visual Science
|February 27, 2001
PubMed
Summary

Visual function is more impaired than pupil function in dominant optic atrophy (DOA). This suggests OPA1 genetic defects impact vision pathways more severely than those controlling pupil reflexes.

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

The ying and yang of idebenone: Not too little, not too much - cell death in NQO1 deficient cells and the mouse retina.

Free radical biology & medicine·2019
Same author

The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy.

Mitochondrion·2017
Same author

Mitochondrial dysfunction in an Opa1(Q285STOP) mouse model of dominant optic atrophy results from Opa1 haploinsufficiency.

Cell death & disease·2016
Same author

A randomized, placebo-controlled trial of the benzoquinone idebenone in a mouse model of OPA1-related dominant optic atrophy reveals a limited therapeutic effect on retinal ganglion cell dendropathy and visual function.

Neuroscience·2016
Same author

Direct ophthalmoscopy should be taught to undergraduate medical students.

Eye (London, England)·2015
Same author

Treatment strategies for inherited optic neuropathies: past, present and future.

Eye (London, England)·2014

Area of Science:

  • Ophthalmology
  • Neuroscience
  • Genetics

Background:

  • Dominant optic atrophy (DOA) is a genetic optic neuropathy.
  • The OPA1 gene is implicated in DOA, affecting optic nerve function.
  • Understanding differential pathway vulnerability is crucial for disease mechanisms.

Purpose of the Study:

  • To compare visual and pupil afferent function in patients with dominant optic atrophy (DOA).
  • To investigate the differential impact of the OPA1 genetic defect on visual versus pupillary pathways.

Main Methods:

  • Patients with DOA and healthy controls underwent visual and pupil perimetry.
  • Testing utilized a modified automated perimeter at five stimulus locations.
  • Visual and pupil deficits were quantified and compared at each location.

Related Experiment Videos

Main Results:

  • Both visual and pupil afferent deficits were observed in DOA patients across all tested locations.
  • Visual deficits were significantly greater than pupil deficits at peripheral locations (P < 0.001).
  • No significant difference in deficit severity was found at the fixation point (P = 0.407).

Conclusions:

  • Pupil function is less affected than visual function in DOA at most tested locations.
  • This indicates retinotectal fibers (pupil reflex) are more resilient to OPA1 defects than retinogeniculate fibers (vision).
  • Findings provide insights into the differential vulnerability of optic nerve pathways in genetic optic neuropathies.