Trafficking mechanisms of extracellular matrix macromolecules: Insights from vertebrate development and human diseases |
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| Affiliation: | 1. Department of Medicine, Division of Genetic Medicine, Vanderbilt University Medical Center, Nashville, TN 37232, USA;2. Department of Cell and Developmental Biology, Vanderbilt University Medical Center, Nashville, TN 37232, USA;1. Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St. Louis, MO;2. Center for Genome Sciences and Systems Biology, Department of Genetics, Washington University School of Medicine, St. Louis, MO;1. Department of Paediatrics, University of Calgary Faculty of Medicine and Alberta Children’s Hospital Research Institute, Calgary, Alberta, Canada;2. Department of Pathology (Neuropathology), University of Calgary Faculty of Medicine and Alberta Children’s Hospital Research Institute, Calgary, Alberta, Canada;3. Department of Pathology (Paediatric), University of Calgary Faculty of Medicine and Alberta Children’s Hospital Research Institute, Calgary, Alberta, Canada;4. Department of Clinical Neurosciences, University of Calgary Faculty of Medicine and Alberta Children’s Hospital Research Institute, Calgary, Alberta, Canada;1. School of Engineering, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8656, Japan;2. Department of Mechanical Science and Bioengineering, Osaka University, 1-1 Machikaneyama, Toyonaka, Osaka 560-0043, Japan;3. Computational Science Research Program, RIKEN, 2-1 Hirosawa, Wako, Saitama 351-0198, Japan;1. School of Life Science, Beijing Institute of Technology, Beijing 100081, P.R.China;2. State Key Laboratory for Biology of Plant Diseases and Insect Pests/Institute of Plant Protection, Chinese Academy of Agricultural Sciences, Beijing 100193, P.R.China |
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| Abstract: | Cellular life depends on protein transport and membrane traffic. In multicellular organisms, membrane traffic is required for extracellular matrix deposition, cell adhesion, growth factor release, and receptor signaling, which are collectively required to integrate the development and physiology of tissues and organs. Understanding the regulatory mechanisms that govern cargo and membrane flow presents a prime challenge in cell biology. Extracellular matrix (ECM) secretion remains poorly understood, although given its essential roles in the regulation of cell migration, differentiation, and survival, ECM secretion mechanisms are likely to be tightly controlled.Recent studies in vertebrate model systems, from fishes to mammals and in human patients, have revealed complex and diverse loss-of-function phenotypes associated with mutations in components of the secretory machinery. A broad spectrum of diseases from skeletal and cardiovascular to neurological deficits have been linked to ECM trafficking. These discoveries have directly challenged the prevailing view of secretion as an essential but monolithic process. Here, we will discuss the latest findings on mechanisms of ECM trafficking in vertebrates. |
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| Keywords: | ECM Collagen secretion Membrane traffic Vertebrate animal models Cartilage and bone |
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