Ryanodine Receptors: Structure, function and dysfunction in clinical disease

✍ Scribed by Alexander Kushnir, A. K. M. M. Mollah, Xander H. T. Wehrens (auth.), Xander H. T. Wehrens, Andrew R. Marks (eds.)

• Publisher: Springer US
• Year: 2005
• Tongue: English
• Leaves: 336
• Series: Developments in Cardiovascular Medicine 254
• Edition: 1
• Category: Library

✦ Synopsis

In recent years, the ryanodine receptor has emerged as a new and very promising target for the treatment of several cardiovascular disorders, including cardiac arrhythmias and heart failure. This volume is the most current publication devoted to the major intracellular calcium-release channel, the ryanodine receptor.

"In this series of brief but informative chapters, the contributions progress from the basic gene family and primary structure, through its 3D structure so far, to its regulation and physiology."

David E. Clapham, MD, PhD
Professor of Neurobiology and Pediatrics
Harvard Medical School

Dr. Xander H.T. Wehrens received his M.D. and Ph.D. degrees from Maastricht University in the Netherlands. His research has mainly concentrated on molecular mechanisms of cardiac arrhythmias, in particular in the setting of inherited arrhythmogenic syndromes and congestive heart failure. This work has led to the development of novel anti-arrhythmic therapies. He is currently a research scientist in the Department of Physiology and Cellular Biophysics at the College of Physicians and Surgeons of Columbia University.

Dr. Andrew R. Marks is the Chair and Professor of the Department of Physiology and Cellular Biophysics at Columbia University College of Physicians and Surgeons. Dr. Marks' research has focused on understanding how macromolecular signaling complexes regulate ion channel function in muscle and non-muscle systems, and on the regulation of vascular smooth muscle proliferation and migration. His work has contributed new understandings of fundamental mechanisms that regulate muscle contraction that have lead to the discovery of molecular defects that contribute to heart failure and fatal cardiac arrhythmias.

✦ Table of Contents

Evolution of the Ryanodine Receptor Gene Family....Pages 1-8
Topology and Transmembrane Organization of Ryanodine Receptors....Pages 9-23
Three-Dimensional Reconstruction of Ryanodine Receptors....Pages 25-34
RYR-DHPR Relationships in Skeletal and Cardiac Muscles....Pages 35-41
The Pore of the Ryanodine Receptor Channel....Pages 43-52
Intra-Molecular Domain-Domain Interaction....Pages 53-65
Regulation of Sarcoplasmic Reticulum Calcium Release by Luminal Calcium....Pages 67-76
Cytosolic Calcium Regulation of Single Ryanodine Receptor Channels....Pages 77-86
Elementary Ca 2+ Release Events: Ryanodine Receptor Ca 2+ Sparks....Pages 87-97
Ca 2+ Release from the Sarcoplasmic Reticulum in Intact Cardiomyocytes....Pages 99-109
Stability and Instability of Ca 2+ Release from the SR....Pages 111-119
Ryanodine Receptors in Smooth Muscle....Pages 121-130
Functions of RYR3 Homologues....Pages 131-140
Knockout Mice Lacking RYR and Junctophilin Subtypes....Pages 141-150
Regulation of Ryanodine Receptor Ca 2+ Release by Macromolecular Complexes....Pages 151-161
RYR1 Modulation by Calmodulin....Pages 163-168
Ryanodine Receptor Function in Inflammation....Pages 169-178
Ryanoids, Receptor Affinity and RYR Channel Subconductance....Pages 179-189
Scorpion Peptides as High-Affinity Probes of Ryanodine Receptor Function....Pages 191-200
Redox Sensing by the Ryanodine Receptors....Pages 201-208
Ryanodine Receptor Dysfunction in the Diabetic Heart....Pages 209-218
Molecular and Clinical Genetics of RYR1 Disorders....Pages 219-227
Pathophysiology of Muscle Disorders Linked to Mutations in the Skeletal Muscle Ryanodine Receptor....Pages 229-242
The Dantrolene Binding Site on RYR1....Pages 243-251
Ryanodine Receptor Dysfunction in Heart Failure and Arrhythmias....Pages 253-261
Stabilization of Ryanodine Receptor as a Novel Therapeutic Strategy Against Heart Failure....Pages 263-272
Ryanodine Receptor Antibodies and Myasthenia Gravis....Pages 273-279

✦ Subjects

Cardiology