Staff Publications

Staff Publications

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    'Staff publications' is the digital repository of Wageningen University & Research

    'Staff publications' contains references to publications authored by Wageningen University staff from 1976 onward.

    Publications authored by the staff of the Research Institutes are available from 1995 onwards.

    Full text documents are added when available. The database is updated daily and currently holds about 240,000 items, of which 72,000 in open access.

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Record number 429422
Title The inflammatory response in Acyl-CoA oxidase 1 deficiency (pseudoneonatal adrenoleukodystrophy)
Author(s) Hajj, H.I. El; Vluggens, A.; Andreoletti, P.; Ragot, K.; Mandard, S.J.; Kersten, A.H.; Waterham, H.R.; Lizard, G.; Wanders, R.J.A.; Reddy, J.K.; Cherkaoui-Malki, M.
Source Endocrinology 153 (2012)6. - ISSN 0013-7227 - p. 2568 - 2575.
DOI https://doi.org/10.1210/en.2012-1137
Department(s) Chair Nutrition Metabolism and Genomics
VLAG
Publication type Refereed Article in a scientific journal
Publication year 2012
Keyword(s) x-linked adrenoleukodystrophy - multiple-sclerosis lesions - nf-kappa-b - peroxisomal disorders - chemokine receptors - microarray analysis - in-vitro - interleukin-1 - cells - gene
Abstract Among several peroxisomal neurodegenerative disorders, the pseudoneonatal adrenoleukodystrophy (P-NALD) is characterized by the acyl-coenzyme A oxidase 1 (ACOX1) deficiency, which leads to the accumulation of very-long-chain fatty acids (VLCFA) and inflammatory demyelination. However, the components of this inflammatory process in P-NALD remain elusive. In this study, we used transcriptomic profiling and PCR array analyses to explore inflammatory gene expression in patient fibroblasts. Our results show the activation of IL-1 inflammatory pathway accompanied by the increased secretion of two IL-1 target genes, IL-6 and IL-8 cytokines. Human fibroblasts exposed to very-long-chain fatty acids exhibited increased mRNA expression of IL-1a and IL-1ß cytokines. Furthermore, expression of IL-6 and IL-8 cytokines in patient fibroblasts was down-regulated by MAPK, p38MAPK, and Jun N-terminal kinase inhibitors. Thus, the absence of acyl-coenzyme A oxidase 1 activity in P-NALD fibroblasts triggers an inflammatory process, in which the IL-1 pathway seems to be central. The use of specific kinase inhibitors may permit the modulation of the enhanced inflammatory status.
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