Peroxisomal Disorders and Regulation of Genes

Abstract

* Foreword H. Galjaard. Why study regulation of genes in inherited disorders? F. Roels. * Variable Expression Of Peroxisomes And Their Disorders. Phenotypic variability (heterogeneity) of peroxisomal disorders H. Mandel, S. Korman. Mulibrey nanism: a novel peroxisomal disorder J. Kallijarvi, et al. Peroxisomes during development and in distinct cell types F. Roels, et al. Tissue-specific expression of two peroxisomal 3-ketoacyl-CoA thiolase genes in wild and PPARalpha-null mice and induction by fenofibrate G. Chevillard, et al. Clinical features and retinal function in patients with adult Refsum syndrome B. Leroy, et al. Is there a phenotype/genotype correlation in peroxisome biogenesis disorders (PBDs)? J. Gartner. Biochemical markers predicting survival in peroxisome biogenesis disorders J. Gootjes, et al. Identification of PEX7 as the second gene involved in Refsum disease D. Van Den Brink, et al. Genetic heterogeneity in Japanese patients with peroxisome biogenesis disorders and evidence for a founder haplotype for the most common mutation in PEX10 gene N. Shimozawa, et al. Disturbances of valine metabolism in patients with peroxisomal biogenesis disorders F. Eyskens, M. Lefevere. Mouse models and genetic modifiers in X-linked adrenoleukodystrophy A. Heinzer, et al. Evidence against the adrenoleukodystrophy-related gene acting as a modifier of X-adrenoleukodystrophy A. Holzinger, et al. Peroxisome mosaics F. Roels, et al. Resolution of the molecular defect in a patient with peroxisomal mosaicism in the liver J. Gootjes, et al. Lessons from knockout mice I: Phenotypes of mice with peroxisome biogenesis disorders M. Baes, P. Van Veldhoven. Lessons from knockout mice II: Mouse models for peroxisomal disorders with singleprotein deficiency J. Berger, et al. * Molecular Mechanisms Of Gene Regulation. DNA methylation and human diseases O. El-Maarri. RNA silencing J. Grabarek. Imprinting M. De Rycke. Histone Modifications-Marks for Gene Expression? A. Imhof. A paradigm for gene regulation: inflammation, NF-kB and PPAR W. Vanden Berghe, et al. * Investigative Techniques. Methods: DNA methylation O. El-Maarri. RNA interference in mammalian systems: A practical approach J. Grabarek, M. Zernicka-Goetz. Histone modifications: methods and techniques A. Imhof. Characterization of the peroxisomal cycling receptor Pex5p import pathway A. Gouveia, et al. Interaction of PEX3 and PEX19 visualized by fluorescence resonance energy transfer (FRET) A. Muntau, et al. * Regulation Of Peroxisome Expression. Gene Regulation of Peroxisomal Enzymes by Nutrients, Hormones and Nuclear Signalling Factors in Animal and Human Species N. Latruffe, et al. Regulation of peroxisomal genes by dehydroepiandosterone and vit D M. Depreter, et al. Effect of DHEA supplementation on fatty acid and hormone levels in patients with X-linked adrenoleukodystrophy J. Assies, et al. Dehydroepiandrosterone induction of the Abcd2 and Abcd3 genes encoding peroxisomal ABC transporters: implications for X-linked adrenoleukodystrophy F. Gueugnon, et al. Phytanic and pristanic acids are naturally occurring ligands A. Zomer, et al. Modifying the peroxisomes by cell & tissue culture: I. Modified peroxisomes in primary hepatocyte cultures M. Depreter, et al. II. Fibroblasts M. Giros, M. Ruiz. III. Peroxisomes and PPAR in cultured neural cells A. Cimini, et al. Pharmacological induction of redundant genes for a therapy