| Identification of a new fatty acid synthesis-transport machinery at the peroxisomal membrane. | |
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MedLine Citation:
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PMID: 22045812 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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X-linked adrenoleukodystrophy (X-ALD) is a complex early-onset neurodegenerative disease characterized by the abnormal accumulation of very long chain fatty acids (VLCFAs) especially in brain white matter, adrenal cortex and testis. Mutations in the gene encoding the peroxisomal ABC half-transporter adrenoleukodystrophy protein (ALDP) are the primary cause of X-ALD. The exact function of peroxisomal ABC half-transporters is still unresolved and defects of ALDP alone are insufficient to explain the wide range of divergent clinical X-ALD phenotypes. 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 activation (FATP4), constituting a so far unknown fatty acid synthesis-transport machinery at the cytoplasmic side of the peroxisomal membrane. This machinery adds knowledge to 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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Authors:
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Merle Hillebrand; Soeren W Gersting; Amelie S Lotz-Havla; Annika Schaefer; Hendrik Rosewich; Oliver Valerius; Ania C Muntau; Jutta Gaertner |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-11-1 |
Journal Detail:
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Title: The Journal of biological chemistry Volume: - ISSN: 1083-351X ISO Abbreviation: - Publication Date: 2011 Nov |
Date Detail:
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Created Date: 2011-11-2 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 2985121R Medline TA: J Biol Chem Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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Georg August University, Germany; |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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