Wolfram syndrome 1 (WFS1) protein expression in retinal ganglion cells and optic nerve glia of the cynomolgus monkey

Hideo Yamamoto, Sabine Hofmann, Duco I. Hamasaki, Hiroko Yamamoto, Pawel Kreczmanski, Christoph Schmitz, Jean Marie Parel, Rainald Schmidt-Kastner

Research output: Contribution to journalArticle

23 Scopus citations

Abstract

Wolfram syndrome (WFS1, OMIM 222300) is a rare genetic disorder associated with multiple organ abnormalities, most prominently optic nerve atrophy and diabetes. Mutations in the WFS1 gene coding for wolframin have been identified. The pathogenesis for optic nerve atrophy remains elusive. We here tested the hypothesis that wolframin is expressed in glial cells of the optic nerve and in retinal ganglion cells in the cynomolgus monkey. Paraffin sections through the retina and optic nerve were examined with immunohistochemistry using affinity-purified antibodies to wolframin. Retinal ganglion cells and optic nerve glial cells were found to be strongly labeled. Dual dysfunction of wolframin in optic nerve glial cells and retinal ganglion cells may explain the progressive optic nerve atrophy in Wolfram syndrome.

Original languageEnglish (US)
Pages (from-to)1303-1306
Number of pages4
JournalExperimental Eye Research
Volume83
Issue number5
DOIs
StatePublished - Nov 2006

Keywords

  • degeneration
  • immunohistochemistry
  • retina
  • Wolfram syndrome

ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems

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    Yamamoto, H., Hofmann, S., Hamasaki, D. I., Yamamoto, H., Kreczmanski, P., Schmitz, C., Parel, J. M., & Schmidt-Kastner, R. (2006). Wolfram syndrome 1 (WFS1) protein expression in retinal ganglion cells and optic nerve glia of the cynomolgus monkey. Experimental Eye Research, 83(5), 1303-1306. https://doi.org/10.1016/j.exer.2006.06.010