Extracellular Matrix Genes provides some of the interesting complexities of the structure of the entire family of extracellular matrix genes. This book illustrates the permanent role that molecular biology and molecular genetics play in the detailed understanding of the normal biology of extracellular matrix. Organized into 10 chapters, this book begins with an overview of the structural and sequence aspects of the entire family of genomic sequences with a view to establishing common functional domains among collagens and their genes. This text then examines the status of the literature concerning human types. III and V. Other chapters consider the FACIT collagen gene family. This book discusses as well the development in understanding the structure of small proteoglycan core proteins and their role in proteoglycan biosynthesis and function as a result of their molecular cloning and expression. The final chapter deals with mutations in collagen genes. This book is a valuable resource for biochemists.

Cells in the developing embryo depend on signals from the extracellular environment to help guide their differentiation. An important mediator in this process is the extracellular matrix – secreted macromolecules that interact to form large protein networks outside the cell. During development, the extracellular matrix serves to separate adjacent cell groups, participates in establishing morphogenic gradients, and, through its ability to interact directly will cell-surface receptors, provides developmental clocks and positional information. This volume discusses how the extracellular matrix influences fundamental developmental processes and how model systems can be used to elucidate ECM function. The topics addressed range from how ECM influences early development as well as repair processes in the adult that recapitulate developmental pathways.

Knowledge of the extracellular matrix (ECM) is essential to understand cellular differentiation, tissue development, and tissue remodeling. This volume of the series “Biology of Extracellular Matrix” provides a timely overview of the structure, regulation, and function of the major macromolecules that make up the extracellular matrix. It covers topics such as collagen types and assembly of collagen-containing suprastructures, basement membrane, fibronectin and other cell-adhesive glycoproteins, proteoglycans, microfibrils, elastin, fibulins and matricellular proteins, such as thrombospondin. It also explores the concept that ECM components together with their cell surface receptors can be viewed as intricate nano-devices that allow cells to physically organize their 3-D-environment. Further, the role of the ECM in human disease and pathogenesis is discussed as well as the use of model organisms in elucidating ECM function.

The evolution of single cells into multicellular organisms was mediated, in large part, by the extracellular matrix. The proteins and glycoconjugates that make up the extracellular matrix provide structural support to cellular complexes, facilitate cell adhesion and migration, and impart mechanical properties that are important for tissue function. Each class of ECM macromolecule has evolved to incorporate distinctive properties that are defined by conserved modules that are mixed together to achieve appropriate function. This volume provides a comprehensive analysis of how the major ECM components evolved over time in order to fill their specific roles found in modern organisms. The major focus is on the structural matrix proteins, matricellular proteins, and more complex ECM structures such as basement membranes. Adhesive proteins and their receptors are also discussed.

Cell Shape: Determinants, Regulation, and Regulatory Role is written to help readers understand cell shape and its regulation. It provides insight into how the shape of a cell is determined at a molecular level. It also discusses how the shape of the cell, as a signal, controls gene expression and modulates intercellular interaction. Divided into four parts encompassing 13 chapters, this volume begins with an overview of the mechanism of shape determination. It then explores how the cytoskeleton, the extracellular matrix, and biochemical force transduction may affect or respond to cell shape control. Other chapters focus on the mechanical modeling of cell shape and the biochemical reactions underlying cell motility. The text also describes the results of the molecular studies that have provided important information on the composition of the cytoskeleton. The final chapter reviews the shape changes that happen during embryogenesis. It also considers how the polarity of the embryo is determined. This book is a valuable resource for biologists, embryologists, and cell biologists.

In the ten-year interval since the first edition of this volume went to press, our knowledge of extracellular matrix (ECM) function and structure has enor mously increased. Extracellular matrix and cell-matrix interaction are now routine topics in the meetings and annual reviews sponsored by cell biology societies. Research in molecular biology has so advanced the number of known matrix molecules and the topic of gene structure and regulation that we won dered how best to incorporate the new material. For example, we deliberated over the inclusion of chapters on molecular genetics. We decided that with judicious editing we could present the recent findings in molecular biology within the same cell biology framework that was used for the first edition, using three broad headings: what is extracellular matrix, how is it made, and what does it do for cells? Maintaining control over the review of literature on the subject of ECM was not always an easy task, but we felt it was essential to production of a highly readable volume, one compact enough to serve the the student as an introduction and the investigator as a quick update on graduate the important recent discoveries. The first edition of this volume enjoyed con hope the reader finds this edition equally useful. siderable success; we D. Hay Elizabeth vii Contents Introductory Remarks 1 Elizabeth D. Hay PART I. WHAT IS EXTRACELLULAR MATRIX? Chapter 1 Collagen T. F. Linsenmayer 1. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 2. The Collagen Molecule . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8 2. 1. Triple-Helical Domain(s) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

Knowledge about the mechanisms of lung development has been growing rapidly, especially with regard to cellular and molecular aspects of growth and differentiation. This authoritative international volume reviews key aspects of lung development in health and disease by providing a comprehensive review of the complex series of cellular and molecular interactions required for lung development. It covers such topics as pulmonary hypoplasia, effects of malnutrition, and pulmaonary angiogenesis. An indispensable reference for all those involved in studying or treating lung disease in neonates and children, the book offers a unique view of the development of this essential organ.

Tissue Repair, Contraction and the Myofibroblast summarizes the latest findings concerning the biology of the myofibroblast, a cell involved in the evolution and contraction of granulation tissue and of fibrotic changes. Coverage shows that the myofibroblast is responsible for the development of hypertrophic scars, pulmonary and renal fibrosis and bronchial asthma. Reviews the cell biology and pathology of the myofibroblast as well as mechanisms of fibrosis evolution in many organs and tissues.

This Atlas of Inherited Retinal Disorders provides a thorough overview of various inherited retinal dystrophies with emphasis on phenotype characteristics and how they relate to the most frequently encountered genes. It also meets the previously unmet needs of PhD students who will benefit from seeing the phenotypes of genes they work on and study. Further, because genetic-testing costs are quite high and spiraling higher, this Atlas will help geneticists familiarize themselves with the candidate gene approach to test patients’ genomes, enabling more cost-efficient testing. This invaluable atlas is organized into eight sections starting with an introduction to the basic knowledge on retinal imaging, followed by diseases listed according to inheritance pattern and disorders with extraocular manifestations grouped by defining features. This structure will be intuitive to clinicians and students studying inherited retinal disorders.