Peroxisomal β-oxidation—A metabolic pathway with multiple functions

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Peroxisomal β-oxidation—A metabolic pathway with multiple functions

Fatty acid degradation in most organisms occurs primarily via the β-oxidation cycle. In mammals, β-oxidation occurs in both mitochondria and peroxisomes, whereas plants and most fungi harbor the β-oxidation cycle only in the peroxisomes. Although several of the enzymes participating in this pathway in both organelles are similar, some distinct physiological roles have been uncovered. Recent adv...

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Peroxisomal β-oxidation in X-linked adrenoleukodystrophy

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-...

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ژورنال

عنوان ژورنال: Biochimica et Biophysica Acta (BBA) - Molecular Cell Research

سال: 2006

ISSN: 0167-4889

DOI: 10.1016/j.bbamcr.2006.08.034