Peroxisomal Disorders and Regulation of Genes

Advances in Experimental Medicine and Biology 544

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Bibliografische Daten
ISBN/EAN: 9780306481741
Sprache: Englisch
Umfang: XIV, 429 S.
Auflage: 1. Auflage 2003
Einband: gebundenes Buch


Inhaltsangabe* 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. Kallijärvi, 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. Gärtner. 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-&kgr;B 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

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