Document Detail

Mitochondrial fatty acid beta-oxidation in the retinal pigment epithelium.
MedLine Citation:
PMID:  12357056     Owner:  NLM     Status:  MEDLINE    
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-13C]-hexadecanoic acid. Acylcarnitine esters were analyzed by tandem mass spectrometry. The activity of LCHAD and carnitine uptake capacity were measured from the cultured cells. Antibodies to the human mitochondrial trifunctional protein (MTP) containing LCHAD activity were used to analyze the expression of the MTP in the cultured RPE cell lysate and in human retinal sections by immunoblotting and immunohistochemistry. Fatty acid oxidation analysis showed normal chain shortening of hexadecanoic acid and production of acetylcarnitine in cultured RPE cells. Immunoblotting revealed expression of the MTP and enzyme assay showed the activity of LCHAD in the RPE cells. RPE cells were also capable of carnitine uptake. Positive labeling to the MTP antibodies was detected in the RPE, photoreceptors, and ganglion cells. The results give strong in vitro evidence for the presence of mitochondrial fatty acid beta-oxidation in RPE cells and the expression of the MTP in the RPE and other layers of the retina. Further studies are required to clarify whether this pathway acts also in vivo in the retina.
Tiina Tyni; Margaret Johnson; Simon Eaton; Morteza Pourfarzam; Richard Andrews; Douglass M Turnbull
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Pediatric research     Volume:  52     ISSN:  0031-3998     ISO Abbreviation:  Pediatr. Res.     Publication Date:  2002 Oct 
Date Detail:
Created Date:  2002-10-01     Completed Date:  2003-03-04     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0100714     Medline TA:  Pediatr Res     Country:  United States    
Other Details:
Languages:  eng     Pagination:  595-600     Citation Subset:  IM    
Department of Neurology, University of Newcastle upon Tyne, UK.
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MeSH Terms
Blotting, Western
Cells, Cultured
Fatty Acids / metabolism*
Mitochondria / metabolism*
Pigment Epithelium of Eye / metabolism*
Reg. No./Substance:
0/Fatty Acids

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