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The Influence of Mitochondrial Dynamics and Function on Retinal Ganglion Cell Susceptibility in Optic Nerve Disease.
Muench, Nicole A; Patel, Sonia; Maes, Margaret E; Donahue, Ryan J; Ikeda, Akihiro; Nickells, Robert W.
Afiliación
  • Muench NA; Department of Ophthalmology and Visual Sciences, University of Wisconsin-Madison, Madison, WI 53706, USA.
  • Patel S; Department of Ophthalmology and Visual Sciences, University of Wisconsin-Madison, Madison, WI 53706, USA.
  • Maes ME; Institute of Science and Technology Austria, 3400 Klosterneuburg, Austria.
  • Donahue RJ; Department of Ophthalmology and Visual Sciences, University of Wisconsin-Madison, Madison, WI 53706, USA.
  • Ikeda A; Boston Children's Hospital, Harvard Medical School, Harvard University, Boston, MA 02115, USA.
  • Nickells RW; Department of Medical Genetics, University of Wisconsin-Madison, Madison, WI 53706, USA.
Cells ; 10(7)2021 06 25.
Article en En | MEDLINE | ID: mdl-34201955
The important roles of mitochondrial function and dysfunction in the process of neurodegeneration are widely acknowledged. Retinal ganglion cells (RGCs) appear to be a highly vulnerable neuronal cell type in the central nervous system with respect to mitochondrial dysfunction but the actual reasons for this are still incompletely understood. These cells have a unique circumstance where unmyelinated axons must bend nearly 90° to exit the eye and then cross a translaminar pressure gradient before becoming myelinated in the optic nerve. This region, the optic nerve head, contains some of the highest density of mitochondria present in these cells. Glaucoma represents a perfect storm of events occurring at this location, with a combination of changes in the translaminar pressure gradient and reassignment of the metabolic support functions of supporting glia, which appears to apply increased metabolic stress to the RGC axons leading to a failure of axonal transport mechanisms. However, RGCs themselves are also extremely sensitive to genetic mutations, particularly in genes affecting mitochondrial dynamics and mitochondrial clearance. These mutations, which systemically affect the mitochondria in every cell, often lead to an optic neuropathy as the sole pathologic defect in affected patients. This review summarizes knowledge of mitochondrial structure and function, the known energy demands of neurons in general, and places these in the context of normal and pathological characteristics of mitochondria attributed to RGCs.
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Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Células Ganglionares de la Retina / Enfermedades del Nervio Óptico / Dinámicas Mitocondriales Límite: Animals / Humans Idioma: En Revista: Cells Año: 2021 Tipo del documento: Article País de afiliación: Estados Unidos

Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Células Ganglionares de la Retina / Enfermedades del Nervio Óptico / Dinámicas Mitocondriales Límite: Animals / Humans Idioma: En Revista: Cells Año: 2021 Tipo del documento: Article País de afiliación: Estados Unidos