Peroxisomal Disorders and Regulation of Genes Book

	Foreword
	Why study regulation of genes in inherited disorders?	1
	Phenotypic variability (heterogeneity) of peroxisomal disorders	9
	Mulibrey nanism: a novel peroxisomal disorder	31
	Peroxisomes during development and in distinct cell types	39
	Tissue-specific expression of two peroxisomal 3-ketoacyl-CoA thiolase genes in wild and PPAP[alpha]-null mice and induction by fenofibrate	55
	Clinical features and retinal function in patients with adult Refsum syndrome	57
	Is there a phenotype/genotype correlation in peroxisome biogenesis disorders (PBDs)?	59
	Biochemical markers predicting survival in peroxisome biogenesis disorders	67
	Identification of PEX7 as the second gene involved in Refsum disease
	Genetic heterogeneity in Japanese patients with peroxisome biogenesis disorders and evidence for a founder haplotype for the most common mutation in PEX10 gene	71
	Disturbances of valine metabolism in patients with peroxisomal biogenesis disorders	73
	Mouse models and genetic modifiers in X-linked adrenoleukodystrophy
	Evidence against the adrenoleukodystrophy-related gene acting as a modifier of X-adrenoleukodystrophy	95
	Peroxisome mosaics
	Resolution of the molecular defect in a patient with peroxisomal mosaicism in the liver	107
	Lessons from knockout mice I: Phenotypes of mice with peroxisome biogenesis disorders	113
	Lessons from knockout mice II: Mouse models for peroxisomal disorders with single protein deficiency	123
	DNA methylation and human diseases	135
	RNA silencing	145
	Imprinting	159
	Histone Modifications - Marks for Gene Expressions?	169
	A paradigm for gene regulation: inflammation, NF-[kappa]B and PPAR	181
	Methods: DNA methylation	197
	RNA interference in mammalian systems: A practical approach	205
	Histone modifications: methods and techniques	217
	Characterization of the peroxisomal cycling receptor Pex5p import pathway	219
	Interaction of PEX3 and PEX19 visualized by fluorescence resonance energy transfer (FRET)	221
	Gene Regulation of Peroxisomal Enzymes by Nutrients, Hormones and Nuclear Signalling Factors in Animal and Human Species	225
	Regulation of peroxisomal genes by dehydroepiandosterone and vit D	237
	Effect of DHEA supplementation on fatty acid and hormone levels in patients with X-linked adrenoleukodystrophy Johanna Assies	243
	Dehydroepiandosterone induction of the Abcd2 and Abcd3 genes encoding peroxisomal ABC transporters: implications for X-linked adrenoleukodystrophy	245
	Phytanic and pristanic acids are naturally occurring ligands	247
	Modifying the peroxisomes by cell & tissue culture: I. Modified peroxisomes in primary hepatocyte cultures	255
	Modifying the peroxisomes by cell & tissue culture: II. Fibroblasts	265
	Modifying the peroxisomes by cell & tissue culture: III. Peroxisomes and PPAR in culture neural cells	271
	Pharmacological induction of redundant genes for a therapy of X-ALD: phenylbutyrate and other compounds
	Peroxisomal fatty acid alpha- and beta-oxidation in health and disease: new insights	293
	Role of phytanoyl-CoA 2-hydroxylase in phytanic acid degradation	303
	Thiamine pyrophospate: an essential cofactor in the mammalian metabolism of 3-methyl-branched fatty acids - implications for thiamine deficiency?	305
	Metabolic regulation of peroxisomal and mitochondrial fatty acid oxidation	307
	Cholesterol biosynthesis and regulation: role of peroxisomes	315
	Peroxisome deficiency does not result in deficiency of enzymes involved in cholesterol biosynthesis	329
	Cholesterol regulates ABCD2 gene expression: implication for X-linked adrenoleuko-dystrophy	331
	Regulations of farnesyl diphosphate synthase gene expression by fatty acids	333
	Plasmalogens, docosahexaenoic acid and neurological disorders	335
	Targeted disruption of ether lipid synthesis in mice	355
	Evaluation of the preventive effect of glyceryl trioleate-trierucate (Lorenzo's oil) therapy in presymtomatic X-linked adrenoleukodystrophy. Results of two concurrent studies
	Author Index	389
	Subject Index	391