Immunohistochemical study of fatty acid metabolism in Muller cells of the retina.

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The nutrient metabolism of Muller cell of the retina, fatty acid β-oxidation system in particular, was attempted by biological and morphological studies. All the enzymes examined were shown to be present in the retina with the amount of protein and gene expression in the retina being about 1/10 of those in the liver. Electron microscopic immunohistochemistry revealed the enzymes to be localized to the mitochondria of Muller cells. The present results are helpful in elucidating the pathogenesis of retinal pigmentary degeneration in the inborn error of metabolism in fatty acid β-oxdation.