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: |
Introduction / 1: |
The Human Complement of ABC Transporters / 2: |
The Core Architecture of an ABC Transporter Comprises Two TMDs and Two NBDs / 3: |