| Contributors |
| Preface |
| General Introduction, Structure and Likely Mechanism of Action of ABC Transport Proteins / Kenneth J. Linton ; Joseph K. Zolnerciks ; Lutz SchmittChapter 1： |
| Introduction / 1： |
| The Human Complement of ABC Transporters / 2： |
| The Core Architecture of an ABC Transporter Comprises Two TMDs and Two NBDs / 3： |
| Structure Analysis and Its Relevance to Molecular Mechanisms / 4： |
| Conformational Changes During the Transport Cycle / 5： |
| The ATP Switch Hypothesis / 6： |
| Future Perspectives / 7： |
| References |
| ABCAl-Mediated HDL Formation: Mechanism and Regulation / Kohjiro Nagao ; Masako Hozoji-Inada ; Yuya Azutna ; Kazumitsu UedaChapter 2： |
| ABCA1 and HDL Formation |
| Structural Feature of ABCA1 |
| Mechanism of apoA-I-Dependent Cholesterol Secretion by ABCA1 |
| Membrane Meso-Domains and ABCA1 Functions |
| SM Content and Functions of ABCA1 |
| Substrate Transport Specificity |
| ApoA-I and Sodium Taurocholate, Lipid Acceptors for ABCA1 and ABCB4 |
| Four-Step Model for HDL Formation / 8： |
| Endocytosis and Recycling of ABCA1 / 9： |
| Degradation of ABCA1 / 10： |
| Regulation of ABCA1 by Interacting Proteins / 11： |
| Perspectives for the Future / 12： |
| Acknowledgments |
| Bile Formation and the Enterohepatic Circulation / Peter H. Dixon ; Catherine WilliamsonChapter 3： |
| The Enterohepatic Circulation |
| ABC Transporters and Bile Formation - The Central Role of ABCB4 and ABCB11 |
| ABCB1 and ABCG2: Deciphering the Role of Human Efflux Proteins in Cellular and Tissue Permeability / Marcos M. Pires ; Dana M. Emmert ; Jean A. Chmielewski ; Christine A. HrycynaChapter 4： |
| Structural Studies of ABCB1 and ABCG2 |
| Physiological Roles of ABCB1 and ABCG2 |
| Drug Bioavailability |
| Disease States Related to ABCB1 and ABCG2 |
| Neurological Disease |
| Viral Disease |
| Multidrug Resistance in Cancer |
| Inhibition of ABCB1 and ABCG2 Transport Activity in the Cancer Clinic |
| ABCA4 and Its Role in the Visual Cycle and Inherited Retinal Degenerative Diseases / Robert S. Molday ; Laurie L. MoldayChapter 5： |
| Overview of the Function of ABCA4 in the Photoreceptor Cells and the Mechanism Responsible for Stargardt Macular Degeneration |
| Localization of ABCA4 in Outer Segments of Photoreceptor Cells |
| Structural Features of ABCA4 |
| Evidence for the Role of ABCA4 as a N-Retinylidene-Phosphatidylethanolamine (N-Ret-PE) Transporter |
| Proposed Role of ABCA4 in the Clearance of N-ret-PE as Part of the Visual Cycle |
| Direction of Substrate Transport by ABCA4 - An Unresolved Issue |
| Inherited Retinal Degenerative Diseases Associated with Mutations in ABCA4 |
| Accumulation of Toxic Retinal Compounds is Responsible for Stargardt Disease |
| Gene and Drug Based Therapeutic Treatments |
| Concluding Remarks |
| The Role of the Antigen Translocation Machinery TAP Adaptive Immunity / Daphne Nikles ; Robert TampéChapter 6： |
| Organization and Function of the PLC |
| Structure and Function of TAP |
| Viral Immune Evasion Strategies Block Peptide-loading |
| The Bare Lymphocyte Syndrome (BLS) |
| Tumor Escape Mechanisms Target TAP |
| Conclusions and Outlook |
| The ABCs of Mitochondrial Metabolic Disorders / Yu Fukuda ; Dagny Ulrich ; John Lynch ; John D. SchuetzChapter 7： |
| ABCB6 |
| ABCB7 |
| ABCB8 |
| ABCB10 |
| Conclusions and Perspectives |
| The Role of ABCC Family Members in the Disposition of Endogenous Compounds and Drugs / Jörg König ; Martin F. Fromm ; Hartmut GlaeserChapter 8： |
| MRP 1 (Gene Symbol ABCC1) |
| MRP2 (Gene Symbol ABCC2) |
| MRP3 (Gene Symbol ABCC3) |
| MRP4 and MRP5 (Gene Symbols ABCC4 and ABCC5) |
| MRP6 (Gene Symbol ABCC6) |
| Summary and Perspective |
| ABC Transporter Mutations in Heritable Skin Diseases: The ABCC6 and ABCA12 Genes / Qiaoli Li ; Qiujie Jiang ; Jouni UittoChapter 9： |
| The Paradigms of Pseudoxanthoma Elasticum and Harlequin Ichthyosis |
| Clinical Implications of Mutation Analysis in PXE and HI |
| CFTR: Understanding the Cause and Influencing the Outcome of a Major Genetic Disease / Tamás Hegedüs ; Martina Gentzsch ; John R. RiordanChapter 10： |
| CFTR Function |
| Structural Properties of CFTR |
| CFTR Synthesis and Assembly |
| Intracellular Trafficking of CFTR |
| Overcoming Cystic Fibrosis Causing Mutations |
| ABCC8: Physiology and Pathology / David Ortiz ; Yumiko Nakamura ; Hiroshi Hashiguchi ; Joseph Bryan ; Lydia Aguilar-BryanChapter 11： |
| Channel Topology and Assembly |
| Dual Regulation of KAXP by Nucleotides |
| Pharmacology |
| KATP Channels in the Pancreatic Islets of Langerhans |
| KATP Channels in Insulin-Secreting β-Cells |
| KATP in Glucagon-Secreting a-Cells |
| KATP in Somatostatin Secreting 8-Cells |
| Summary of Intra-Islet Regulation |
| Disease States |
| Hyperinsulinemic Hypoglycemia of Infancy |
| Neonatal Diabetes Mellitus |
| Diagnosis and Implications for Therapy / 13： |
| The ABCD Subfamily: Peroxisomal Transporters in Health and Disease / Aurora Pujol ; Patrick AubourgChapter 12： |
| X-Linked Adrenoleukodystrophy (X-ALD): A Complex Disease Due to Loss of Function of ABCD 1 Gene |
| The ABCs of Peroxisomes |
| Fatty Acid Transport Across the Peroxisomal Membrane: The Role of ABCD-Family Transporters |
| The ABCDs of Baker's Yeast |
| The ABCDs of Mammals |
| The Function of ABCD 1 (ALDP) Transporter |
| Do Mammalian Peroxisomal ABCD Half-Transporter Act as Homo- or Heterodimers? |
| ABCD2, ABCD3 and ABCD4 Transporters and Their Overlapping Roles in Fatty Acid Metabolism |
| Inducible Expression of the Mammalian ABCD Proteins and Relevance for X-ALD |
| Conclusion |
| ABCG5/8 and Its Role in Sterol Homeostasis / Ronald P J. Oude Elferink ; Coen C. PaulusmaChapter 13： |
| The ABC Transporter Couple ABCG5/ABCG8 |
| Sitosterolemia |
| Intestinal Sterol Absorption and Excretion |
| Biliary Cholesterol Secretion |
| The Role of Bile in Cholesterol Homeostasis |
| ABCG5/8 and Gallstones |
| Hot Topics, Recent Advances and Novel Approaches for Clinical Intervention / Irwin M. Arias ; Saskia W. C. van Mil ; Heidrun Potschka ; Alan S. Verkman ; Uta Griesenbach ; Eric W. F. W. Alton ; Balazs Sarkadi ; Gergely SzakacsChapter 14： |
| The ABC of Hepatobiliary ABC-Transporter Regulation by Nuclear Receptors |
| Targeting Regulation of ABC Transporters |
| Small-Molecule Corrector Therapy for Cystic Fibrosis |
| Gene Therapy for Cystic Fibrosis |
| The Role of ABC Multidrug Transporters in Normal and Cancer Stem Cells |
| Index |
| Contributors |
| Preface |
| General Introduction, Structure and Likely Mechanism of Action of ABC Transport Proteins / Kenneth J. Linton ; Joseph K. Zolnerciks ; Lutz SchmittChapter 1： |
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