Mitochondrial fatty acid beta-oxidation in the retinal pigment epithelium.

  title={Mitochondrial fatty acid beta-oxidation in the retinal pigment epithelium.},
  author={Tiina A Tyni and Margaret B Johnson and Simon Eaton and Morteza Pourfarzam and Richard Andrews and Douglass Matthew Turnbull},
  journal={Pediatric research},
  volume={52 4},
Pigmentary retinopathy is an important feature of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, a disorder of mitochondrial fatty acid beta-oxidation. Pathogenesis of this complication remains unknown. The retinal pigment epithelium (RPE) is affected early in this retinopathy. We wanted to determine whether there is evidence of mitochondrial fatty acid beta-oxidation in the RPE cells. Fatty acid oxidation was measured from cultured porcine RPE cells by incubating them with [U… CONTINUE READING


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