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Maculopapillary Bundle Degeneration in Optic Neuropathies.

Darius W Lambiri1,2, Leonard A Levin3,4,5

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Maculopapillary bundle (MPB) degeneration, common in optic neuropathies, is linked to mitochondrial dysfunction. New research shows MPB involvement in early glaucoma, distinct from other mitochondrial optic neuropathies.

Keywords:
Dominant optic atrophyGlaucomaLeber hereditary optic neuropathyMaculopapillary bundleMitochondrial dysfunctionOptic neuropathies

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Area of Science:

  • Neuroscience
  • Ophthalmology
  • Cell Biology

Background:

  • Maculopapillary bundle (MPB) degeneration is a key feature across various optic neuropathies.
  • Conditions like nutritional/toxic optic neuropathies, Leber hereditary optic neuropathy (LHON), and dominant optic atrophy (DOA) exhibit MPB-selective degeneration.
  • Despite diverse etiologies, these disorders share a common pathway: compromised mitochondrial homeostasis affecting metabolism, biogenesis, and protein synthesis.

Purpose of the Study:

  • To comprehensively review the role of the MPB in optic neuropathies.
  • To emphasize the critical role of mitochondrial mechanisms in the pathogenesis of these conditions.
  • To synthesize current clinical and molecular research on primary MPB disorders.

Main Methods:

  • Review of optical coherence tomography (OCT) studies characterizing retinal nerve fiber layer changes.
  • Synthesis of clinical and molecular research on primary MPB disorders.
  • Analysis of proposed mechanisms, including reactive oxygen species (ROS) generation.

Main Results:

  • OCT studies reveal characteristic RNFL changes in mitochondrial optic neuropathies, including selective temporal thinning.
  • Mitochondrial dysfunction, leading to ROS generation, is a proposed mechanism for selective MPB atrophy.
  • Recent findings indicate MPB degeneration occurs in early glaucoma, with distinct patterns compared to mitochondrial diseases.

Conclusions:

  • Mitochondrial dysfunction is a central mechanism in MPB degeneration across various optic neuropathies.
  • MPB degeneration in glaucoma presents unique characteristics, challenging previous assumptions.
  • Further in-vivo and in-vitro studies are needed to validate the role of ROS in MPB-selective optic neuropathies.