Mouse Models of Optic Atrophy in the MICRON Literature

About the MICRON Research Literature

These research pages organize published studies using MICRON imaging systems by animal species and the disease or condition investigated.

All publication counts and figures reflect the complete indexed research collection.

Explore related studies through the MICRON Research Hubs or search the complete publication collection in the reSEARCH portal.

Understanding Optic Atrophy

Optic atrophy is a sign rather than a disease in its own right: the pale optic disc left once axons have been lost from the optic nerve, with thinning of the retinal nerve fiber and ganglion cell layers alongside it.

It records the loss and not what produced it, so an atrophic disc is a finding that has still to be explained. Causes recognized in people include hereditary, inflammatory, ischemic, compressive, toxic and traumatic ones, with several inherited diseases carrying the sign in their clinical names.

It is also a late finding, since what it records has already happened, which is why the informative period in both clinic and laboratory is the one before the disc pales.

Optic Atrophy in Experimental Research

What an animal supplies is access to that period: the cause can be fixed by construction, as a genetic lesion or as an injury delivered at a chosen time and site.

The same eye can then be followed while the loss accumulates:

  • By cross-sectional imaging of the inner retinal layers
  • By electrophysiology
  • By behavioral measures of vision
  • With the nerve and the surviving ganglion cell population available for counting afterwards

What an animal does not supply is the way the sign is read in people, which rests on an examiner's judgment of disc color set against a patient's account of their vision, nor the central field loss that characterizes several of the inherited optic atrophies, because that pattern follows the fibers serving the macula, and a mouse retina has no macula and so no papillomacular bundle in which it could appear.

Mouse Models of Optic Atrophy

The mouse carries most of this work.

Transgenic and knockout lines allow a pathway to be removed or overexpressed, and cost, availability, short generation time and a large body of background data reinforce the choice.

A genetic manipulation constrains rather than isolates a mechanism: developmental compensation and pleiotropy remain possible.

Published MICRON Research: Optic Atrophy in Mice

This page lists 10 papers from the MICRON literature, published 2012 to 2026, that study optic atrophy in mice.

Of these publications:

  • 10 studies used mice as the primary optic atrophy disease model.

Scope of the literature collection: Publication counts reflect studies identified and indexed in the MICRON research corpus. This collection is not intended to represent an exhaustive review of all published optic atrophy research.

Which imaging and experimental techniques are used in these studies?

Technique counts are paper-level co-occurrences and do not necessarily indicate that a technique was applied to the focal species or focal disease model.

Capabilities and Limitations of Imaging Techniques

A summary of what each imaging technique listed above can measure, the information it provides, and its limitations in research applications.

Electroretinography

Records the retina's electrical response to light, giving component-sensitive information about outer- and inner-retinal function. It measures function directly, which no structural method does. Full-field ERG sums across the whole retina, so a small lesion can leave the trace unchanged; focal and multifocal variants trade signal amplitude for spatial resolution.

Optical Coherence Tomography

Optical coherence tomography provides high-resolution, cross-sectional images of ocular tissues in the living eye, allowing researchers to visualize structures at different depths. OCT is particularly useful for measuring tissue thickness, identifying structural abnormalities, and assessing the location and extent of tissue changes.

As a non-invasive imaging technique, OCT enables repeated measurements in the same eye over time, supporting longitudinal studies of disease progression and treatment response. Although OCT provides detailed structural information, it does not directly measure cellular activity or visual function. Tissue that appears structurally normal may still have functional abnormalities, making OCT a valuable companion to functional assessments such as electroretinography (ERG).

Topical Ophthalmic Drug Delivery

A compound applied as drops to the ocular surface. It is non-invasive and repeatable, so it supports frequent dosing without a procedure for each dose. What the route has to establish depends on the target: for an ocular-surface target, residence time and penetration into the surface tissue are the main barriers; for an intraocular anterior-segment target, the compound must cross ocular-surface and corneal or conjunctival barriers and reach an intraocular concentration sufficient for the experiment; for a posterior-segment target, additional ocular barriers separate the surface from the target and posterior-segment exposure is harder to establish.

Fundus Fluorescein Angiography

Fundus fluorescein angiography uses an injected fluorescent dye to visualize blood flow through the retinal and choroidal circulation. Sequential images show how vessels fill, identify areas of reduced or absent perfusion, and reveal dye leakage associated with vascular abnormalities.

This technique is particularly useful for evaluating vascular perfusion, blood-retinal barrier integrity, and abnormal vessel growth. Fluorescein angiography primarily shows dye distribution rather than the surrounding retinal tissue. It provides a two-dimensional (en face) view in which vessels at different depths may overlap. Cross-sectional and cellular structural details require other imaging methods, such as optical coherence tomography (OCT) or optical coherence tomography angiography (OCT-A). Vascular abnormalities that do not affect dye filling or produce leakage may also remain undetected.

Optokinetic / Optomotor Response

Measures a reflexive head or eye movement tracking a moving pattern, giving a rapid, training-free behavioral estimate of spatial vision and contrast sensitivity in an animal that cannot report them. It is driven largely by reflex circuitry and reports on the visual pathway as a whole, so a deficit it detects still has to be localized by a structural or electrophysiological method, or separated from cortical processing by an operant task.

AAV-Mediated Ocular Gene Delivery

An adeno-associated viral vector delivers genetic material to ocular cells, and a single administration can support long-lived transgene expression. That persistence is useful for gene augmentation and sustained therapeutic-protein expression; AAV can also deliver gene-silencing or genome-editing cargo, where long-lived expression of the editing machinery may be unnecessary or undesirable. Capsid and route of delivery jointly determine which cells are reached, so different delivery routes and vector designs give different distributions.

Intravitreal Injection / Delivery

Injection into the vitreous places a substance directly into the vitreous at a known administered dose and chosen time, reducing but not eliminating systemic exposure. The injectate may be a therapeutic compound, cell preparation or viral vector, or an agent used to create the model, such as a toxin, vasoactive or excitotoxic compound, or infectious inoculum. The route bypasses surface-delivery barriers and provides direct access to the vitreous and posterior segment, but the concentration reached in a particular ocular tissue depends on distribution and clearance and is not fixed by the injected dose alone. It is invasive and discrete: each repeat dose or challenge requires another injection, and vehicle- or sham-injected controls can help separate effects of the injectate from effects of the procedure.

CRISPR-Based Ocular Gene Editing

Uses a programmable genome-editing system to alter DNA at an endogenous locus rather than adding a separate replacement copy. Depending on the editor and design, it can disrupt, correct or install a sequence, either therapeutically or to create an experimental model. Once a genomic sequence change is established, the edit itself cannot be withdrawn like a dose; where the edit is present from conception, the resulting phenotype also reflects its effects throughout development. Off-target sequence changes are a separate question that structural imaging does not establish.

Ocular Blast Injury

Experimental ocular blast models expose the eye, or in some paradigms the head with ocular outcomes measured, to a controlled overpressure wave at a known time. Peak pressure, waveform and impulse, exposure number, orientation and interval help determine the loading delivered to the eye. A blast wave can injure multiple ocular compartments, including anterior structures, retina and optic nerve, although the pattern and severity depend on the exposure paradigm and the animal. Eye-directed paradigms that shield the head and body are used to isolate primary blast effects on the eye; head-directed or less-confined paradigms may also involve central nervous-system and other traumatic-brain-injury mechanisms and should not be treated as equivalent. Because the exposure is known, interventions can be tested against it and sham exposures can help distinguish effects of the pressure wave from handling or the experimental procedure. Apparatus design, waveform, positioning and shielding materially affect the delivered exposure, so nominal pressure values or exposure counts should not be assumed directly comparable across laboratories or equivalent to human blast exposure without physical characterization.

Which MICRON systems and add-ons appear?

Which MICRON systems and add-ons appear?
Instrument Papers
Image-Guided OCT 5
MICRON III 5
MICRON IV 5
Insight Software 3
MICRON II 1

Which strains and genetic lines are used?

Which strains and genetic lines are used?
Strain Papers Reported as
C57BL/6J 6 C57/Bl6J, C57Bl/6J
129S-Fdxrm1J 1
129S6/SvEvTac × C57BL/6 1
B6 1
BALB/c 1
C3HeB/FeJ-OPA1c.1065+5G>A 1

Which other species appear alongside?

Which other species appear alongside?
Species Papers
Cynomolgus Macaque 1

What other conditions are recorded on the same papers?

What other conditions are recorded on the same papers?
Disease Papers
Retinal Ganglion Cell Degeneration 7
Optic Neuropathy (General) 5
Retinal Degeneration (General) 3
Cataract (General) 2
Dominant Optic Atrophy 2
Retinal Detachment 2
Retinal Pigment Epithelium Dysfunction 2
Corneal Neovascularization 1
Corneal Opacity / Scarring 1
Glaucoma (General) 1
Normal-Tension Glaucoma 1

The general term records an accepted mention of optic atrophy without a subtype qualifier. It is not a roll-up: a paper carrying a subtype may or may not also carry the general term, depending on whether an unqualified mention of the family is also recorded elsewhere in the paper.

Which MICRON configurations appear in this work?

Configurations recorded in these papers, and the techniques recorded alongside them.

Which MICRON configurations appear in this work?
MICRON configuration Papers Most frequent techniques recorded alongside
Image-Guided OCT 5 Electroretinography (5), Optical Coherence Tomography (4), Fundus Fluorescein Angiography (2)
MICRON III 5 Electroretinography (5), Optical Coherence Tomography (3), Topical Ophthalmic Drug Delivery (3)
MICRON IV 5 Optical Coherence Tomography (4), Electroretinography (4), Topical Ophthalmic Drug Delivery (3)
Insight Software 3 Electroretinography (3), Optical Coherence Tomography (2), Fundus Fluorescein Angiography (1)
MICRON II 1 Electroretinography (1), Fundus Fluorescein Angiography (1), Optical Coherence Tomography (1)

The papers

The papers
Year Title Journal Role of focal species MICRON products Links
2026 WFS1 gene delivery rescues visual function in a mouse model of Wolfram syndrome. Acta Neuropathologica Communications Disease model MICRON III DOI · reSEARCH
2024 Mutation of CRYAB encoding a conserved mitochondrial chaperone and antiapoptotic protein causes hereditary optic atrophy. JCI Insight Disease model Image-Guided OCT, MICRON III, MICRON IV DOI · reSEARCH
2023 In Vivo Efficacy and Safety Evaluations of Therapeutic Splicing Correction Using U1 snRNA in the Mouse Retina. Cells Disease model MICRON III DOI · reSEARCH
2023 MCT1-dependent energetic failure and neuroinflammation underlie optic nerve degeneration in Wolfram syndrome mice. eLife Disease model Image-Guided OCT, Insight Software, MICRON IV DOI · reSEARCH
2022 METTL23 mutation alters histone H3R17 methylation in normal-tension glaucoma. The Journal of Clinical Investigation Disease model Image-Guided OCT, Insight Software, MICRON IV DOI · reSEARCH
2020 Integrated analysis of the molecular pathogenesis of FDXR-associated disease. Cell Death & Disease Disease model Image-Guided OCT, Insight Software, MICRON IV DOI · reSEARCH
2016 Retinal Dystrophy and Optic Nerve Pathology in the Mouse Model of Mucolipidosis IV. The American Journal of Pathology Disease model Image-Guided OCT, MICRON III DOI · reSEARCH
2016 Eye-Directed Overpressure Airwave-Induced Trauma Causes Lasting Damage to the Anterior and Posterior Globe: A Model for Testing Cell-Based Therapies. Journal of Ocular Pharmacology and Therapeutics Disease model MICRON IV DOI · reSEARCH
2014 Impairment of visual function and retinal ER stress activation in Wfs1-deficient mice. PLoS ONE Disease model MICRON III DOI · reSEARCH
2012 Preservation of cone photoreceptors after a rapid yet transient degeneration and remodeling in cone-only Nrl-/- mouse retina. The Journal of Neuroscience Disease model MICRON II DOI · reSEARCH

Browse all hubs at MICRON Research Hubs. The full corpus is searchable in the reSEARCH portal.

Last built 2026-11-10 from content tag hubs-content-2026-11-10 (ae06cfc); fact-sheet run sheets-20261110T111119Z. About these hubs Back to top