Identification of a new fatty acid synthesis-transport machinery at the peroxisomal membrane |
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Authors: | Hillebrand Merle Gersting Søren W Lotz-Havla Amelie S Schäfer Annika Rosewich Hendrik Valerius Oliver Muntau Ania C Gärtner Jutta |
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Affiliation: | Department of Pediatrics and Pediatric Neurology, Faculty of Medicine, Georg August University, 37075 G?ttingen, Germany. |
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Abstract: | The neurodegenerative disease X-linked adrenoleukodystrophy (X-ALD) is characterized by the abnormal accumulation of very long chain fatty acids. Mutations in the gene encoding the peroxisomal ATP-binding cassette half-transporter, adrenoleukodystrophy protein (ALDP), are the primary cause of X-ALD. To gain a better understanding of ALDP dysfunction, we searched for interaction partners of ALDP and identified binary interactions to proteins with functions in fatty acid synthesis (ACLY, FASN, and ACC) and activation (FATP4), constituting a thus far unknown fatty acid synthesis-transport machinery at the cytoplasmic side of the peroxisomal membrane. This machinery adds to the knowledge of the complex mechanisms of peroxisomal fatty acid metabolism at a molecular level and elucidates potential epigenetic mechanisms as regulatory processes in the pathogenesis and thus the clinical course of X-ALD. |
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Keywords: | ABC Transporter Bioluminescence Resonance Energy Transfer (BRET) Fatty Acid Metabolism Neurodegenerative Diseases Peroxisomes ACLY ALDP FASN PMP70 X-ALD |
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