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A Review of Mitochondrial Optic Neuropathies: From Inherited to Acquired Forms.


ABSTRACT: In recent years, the term mitochondrial optic neuropathy (MON) has increasingly been used within the literature to describe a group of optic neuropathies that exhibit mitochondrial dysfunction in retinal ganglion cells (RGCs). Interestingly, MONs include genetic aetiologies, such as Leber hereditary optic neuropathy (LHON) and dominant optic atrophy (DOA), as well as acquired aetiologies resulting from drugs, nutritional deficiencies, and mixed aetiologies. Regardless of an inherited or acquired cause, patients exhibit the same clinical manifestations with selective loss of the RGCs due to mitochondrial dysfunction. Various novel therapies are being explored to reverse or limit damage to the RGCs. Here we review the pathophysiology, clinical manifestations, differential diagnosis, current treatment, and promising therapeutic targets of MON.

SUBMITTER: Pilz YL 

PROVIDER: S-EPMC5595256 | biostudies-literature | 2017 Oct - Dec

REPOSITORIES: biostudies-literature

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A Review of Mitochondrial Optic Neuropathies: From Inherited to Acquired Forms.

Pilz Yasmine L YL   Bass Sherry J SJ   Sherman Jerome J  

Journal of optometry 20161228 4


In recent years, the term mitochondrial optic neuropathy (MON) has increasingly been used within the literature to describe a group of optic neuropathies that exhibit mitochondrial dysfunction in retinal ganglion cells (RGCs). Interestingly, MONs include genetic aetiologies, such as Leber hereditary optic neuropathy (LHON) and dominant optic atrophy (DOA), as well as acquired aetiologies resulting from drugs, nutritional deficiencies, and mixed aetiologies. Regardless of an inherited or acquired  ...[more]

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