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Updated: Apr 11, 2026

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Effects of the jimpy mutation on mouse retinal structure and function.

Anahit Hovhannisyan1,2, Boris Benkner1, Antje Biesemeier3

  • 1Retinal Circuits and Optogenetics, Center for Integrative Neuroscience, University of Tübingen, 72076, Tübingen, Germany.

The Journal of Comparative Neurology
|May 27, 2015
PubMed
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The Jimpy mouse, with a mutation affecting proteolipid protein (PLP1), shows reduced myelination in the optic nerve. This impacts retinal ganglion cell function, particularly at lower light levels, but has minimal effects on overall retinal structure.

Area of Science:

  • Neuroscience
  • Genetics
  • Ophthalmology

Background:

  • The Jimpy mouse model exhibits a point mutation in the proteolipid protein gene (plp1).
  • This mutation causes protein misfolding, leading to oligodendrocyte death and myelin destruction in the central nervous system (CNS).
  • The impact of developmental hypomyelination on neural function, particularly in the retina, remains largely uncharacterized.

Purpose of the Study:

  • To investigate the effects of absent optic nerve myelination during development on retinal morphology and function in Jimpy mutant mice.
  • To assess visual system function using optokinetic reflex measurements.
  • To analyze retinal cellular structure and ganglion cell activity.

Main Methods:

  • Optokinetic reflex testing to evaluate visual function.
Keywords:
AB_10000340AB_11180865AB_141607AB_141788AB_2079751AB_2252787AB_2307342AB_2337959AB_2341143AB_305869AB_397879RRIDs: AB_2341144demyelinationoligodendrocytesoptic nerveproteolipid proteinretina

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  • PLP1 antibody staining and reverse transcriptase-polymerase chain reaction (RT-PCR) to assess plp1 expression and myelination.
  • Microelectrode array recordings to measure retinal ganglion cell activity.
  • Morphological analysis including axon counting, ultrastructure, and retinal layer thickness.
  • Immunohistochemistry using cell type-specific markers.

    • Main Results:

    • Jimpy mice possess a generally functional visual system, despite significant hypomyelination of the optic nerve axons.
    • No significant changes were observed in optic nerve axon count or ultrastructure.
    • Reduced stimulus-evoked activity in retinal ganglion cells was detected at mesopic light levels.
    • Retinal morphology was largely unaffected, though some cell types (rod bipolar and horizontal cells) showed increased numbers in the inner nuclear layer.

    Conclusions:

    • The Jimpy mutation severely impairs myelination of retinal ganglion cell axons.
    • Despite significant myelination deficits in the optic nerve, retinal function is moderately affected, with notable changes in ganglion cell activity under specific light conditions.
    • The study highlights a complex relationship between optic nerve myelination and retinal function, with some compensatory mechanisms potentially at play.