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Peroxisomal Disorders and Regulation of Genes

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Cover of 'Peroxisomal Disorders and Regulation of Genes'

Table of Contents

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    Book Overview
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    Chapter 1 Why Study Regulation of Genes in Inherited Disorders?
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    Chapter 2 Phenotypic variability (heterogeneity) of peroxisomal disorders.
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    Chapter 3 Mulibrey Nanism - a Novel Peroxisomal Disorder
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    Chapter 4 Peroxisomes during Development and in Distinct Cell Types
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    Chapter 5 Tissue-specific Expression of Two Peroxisomal 3-ketoacyl-CoA Thiolase Genes in Wild and PPARα-null Mice and Induction by Fenofibrate
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    Chapter 6 Clinical Features & Retinal Function In Patients With Adult Refsum Syndrome
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    Chapter 7 Is there a Phenotype/Genotype Correlation in Peroxisome Biogenesis Disorders (PBDs)?
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    Chapter 8 Biochemical Markers Predicting Survival in Peroxisome Biogenesis Disorders
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    Chapter 9 Identification of PEX7 as the second gene involved in Refsum disease.
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    Chapter 10 Genetic heterogeneity in Japanese patients with peroxisome biogenesis disorders and evidence for a founder haplotype for the most common mutation in PEX10 gene.
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    Chapter 11 Disturbances of Valine Metabolism in Patients with Peroxisomal Biogenesis Disorders
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    Chapter 12 Mouse Models and Genetic Modifiers in X-Linked Adrenoleukodystrophy
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    Chapter 13 Evidence against the Adrenoleukodystrophy-related Gene acting as a Modifier of X-adrenoleukodystrophy
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    Chapter 14 Peroxisome Mosaics
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    Chapter 15 Resolution of the Molecular Defect in a Patient with Peroxisomal Mosaicism in the Liver
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    Chapter 16 Lessons from Knockout Mice. I: Phenotypes of Mice with Peroxisome Biogenesis Disorders
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    Chapter 17 Lessons from Knockout Mice II: Mouse Models for Peroxisomal Disorders with Single Protein Deficiency
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    Chapter 18 DNA Methylation and Human Diseases
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    Chapter 19 RNA Silencing
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    Chapter 20 Imprinting
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    Chapter 21 Histone Modifications-Marks for Gene Expression?
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    Chapter 22 A Paradigm for Gene Regulation: Inflammation, NF-κB and PPAR
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    Chapter 23 Methods: DNA methylation.
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    Chapter 24 RNA interference in mammalian systems--a practical approach.
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    Chapter 25 Histone Modifications:Methods and Techniques
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    Chapter 26 Characterization of the Peroxisomal Cycling Receptor Pex5p Import Pathway
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    Chapter 27 Interaction of PEX3 and PEX19 visualized by fluorescence resonance energy transfer (FRET).
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    Chapter 28 Gene Regulation of Peroxisomal Enzymes by Nutrients, Hormones and Nuclear Signalling Factors in Animal and Human Species
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    Chapter 29 Regulation of Peroxisomal Genes by DHEA and Vitamin D
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    Chapter 30 Effect of Dehydroepiandrosterone (DHEA) Supplementation on Fatty Acid and Hormone Levels in Patients with X-linked Adrenoleukodystrophy
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    Chapter 31 Dehydroepiandrosterone Induction of the Abcd2 and Abcd3 Genes encoding peroxisomal ABC Transporters
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    Chapter 32 Phytanic and pristanic acid are naturally occuring ligands.
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    Chapter 33 Modified Peroxisomes in Primary Hepatocyte Cultures
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    Chapter 34 Modifying the Peroxisomes by Cell & Tissue Culture
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    Chapter 35 Peroxisomes and PPARs in Cultured Neural Cells
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    Chapter 36 Pharmacological Induction of Redundant Genes for a Therapy of X-ALD
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    Chapter 37 Peroxisomal Fatty Acid Alpha-and Beta-Oxidation in Health and Disease: New insights
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    Chapter 38 Role of phytanoyl-CoA 2-hydroxylase in phytanic acid metabolism.
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    Chapter 39 Thiamine Pyrophosphate: an essential Cofactor in the Mammalian Metabolism of 3-methyl-branched Fatty Acids
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    Chapter 40 Metabolic Regulation of Peroxisomal and Mitochondrial Fatty Acid Oxidation
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    Chapter 41 Cholesterol Biosynthesis and Regulation: Role of Peroxisomes
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    Chapter 42 Peroxisome Deficiency Does Not Result in Deficiency of Enzymes involved in Cholesterol Biosynthesis
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    Chapter 43 Cholesterol regulates ABCD2 Gene Expression: implications for X-linked Adrenoleukodstrophy
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    Chapter 44 Regulation of Farnesyl Diphosphate Synthase Gene Expression by Fatty Acids
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    Chapter 45 Plasmalogens, Docosahexaenoic Acid and Neurological Disorders
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    Chapter 46 Targeted Disruption Of Ether Lipid Synthesis In Mice
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    Chapter 47 Evaluation of the Preventive Effect of Glyceryl Trioleate-Trierucate (“Lorenzo’s Oil”) Therapy in X-Linked Adrenoleukodystrophy: Results of Two Concurrent Trials
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Title
Peroxisomal Disorders and Regulation of Genes
Published by
Springer US, December 2012
DOI 10.1007/978-1-4419-9072-3
ISBNs
978-1-4613-4782-8, 978-1-4419-9072-3
Editors

Roels, Frank, Baes, Myriam, Bie, Sylvia

Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 5 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 5 100%

Demographic breakdown

Readers by professional status Count As %
Professor 1 20%
Lecturer > Senior Lecturer 1 20%
Other 1 20%
Student > Ph. D. Student 1 20%
Librarian 1 20%
Other 0 0%
Readers by discipline Count As %
Medicine and Dentistry 4 80%
Chemistry 1 20%