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Peroxisomal β-oxidation in X-linked adrenoleukodystrophy 1 Impaired Very Long-Chain Acyl-CoA β-Oxidation in Human X-ALD Fibroblasts Is a Direct Consequence of ABCD 1 Transporter Dysfunction *
| Content Provider | Semantic Scholar |
|---|---|
| Author | Wiesinger, Christoph Kunze, Markus Regelsberger, Günther Forss-Petter, Sonja Berger, Johannes |
| Copyright Year | 2013 |
| Abstract | Background: ABCD1 is a peroxisomal ABC transporter whose dysfunction causes X-linked adrenoleukodystrophy (X-ALD). Results: β-Oxidation of C26:0 as well as C22:0 acyl-CoA esters is impaired in X-ALD. ABCD3 accounts for residual β-oxidation activity in XALD fibroblasts. Conclusion: ABCD1 mediates very long-chain acyl-CoA ester β-oxidation without need for additional re-esterification by an acyl-CoA synthetase. Significance: Proof of deficient acyl-CoA ester βoxidation in X-ALD. |
| File Format | PDF HTM / HTML |
| Alternate Webpage(s) | http://www.jbc.org/content/early/2013/05/13/jbc.M112.445445.full.pdf |
| Language | English |
| Access Restriction | Open |
| Content Type | Text |
| Resource Type | Article |