By Jeffrey H. Miner
Basement Membranes makes a speciality of really good extracellular matrices that supply the scaffolds used and required by way of cells to arrange themselves into tissues and organs. As basement membranes were proven to be faulty in different genetic and bought ailments and to give a contribution to the microenvironment of either tumor cells and stem cells, this e-book offers a view of particular basement membrane elements and their roles in improvement and sickness, all written and commented on in chapters written through leaders within the basement membrane field.
- Written by means of best specialists within the basement membrane field
- Contains unique fabric, either textual and illustrative, that are supposed to turn into a truly suitable reference material
- Presents fabric in a truly finished manner
- Ideal for either researchers within the box and basic readers who will locate correct and up to date info
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Kreidberg, J. , & Miner, J. H. (2011). Dystroglycan does not contribute signiﬁcantly to kidney development or function, in health or after injury. American Journal of Physiology Renal Physiology, 300, F811eF820. , Brown, S. , Sewry, C. , & Muntoni, F. (2005). Congenital muscular dystrophy: molecular and cellular aspects. Cellular and Molecular Life Sciences, 62, 809e823. , & Engel, J. (1991). Binding and calcium-induced aggregation of laminin onto lipid bilayers. Journal of Biological Chemistry, 266, 19047e19052.
P. (1995). The renal glomerulus of mice lacking s-laminin/laminin beta 2: nephrosis despite molecular compensation by laminin beta 1. Nature Genetics, 10, 400e406. , Sirkowski, E. , Scherer, S. , et al. (2005). Both laminin and schwann cell dystroglycan are necessary for proper clustering of sodium channels at nodes of Ranvier. Journal of Neuroscience, 25, 9418e9427. , et al. (2004). Molecular analysis of laminin n-terminal domains mediating self-interactions. Journal of Biological Chemistry, 279, 44505e44512.
Importantly, the animal models have been indispensable tools for the preclinical development of new therapeutic approaches for laminin-a2 chain deﬁciency, highlighting a number of important disease driving mechanisms that can be targeted by pharmacological approaches. In this chapter, I will describe laminin-211 and discuss the cellular and molecular pathophysiology of MDC1A as well as progression toward development of treatment. 1. INTRODUCTION Each skeletal muscle cell is surrounded by a basement membrane that is directly linked to the sarcolemma (muscle cell membrane).