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      Fell‐Muir Lecture: Fibrillin microfibrils: structural tensometers of elastic tissues?

      review-article
      1 ,
      International Journal of Experimental Pathology
      John Wiley and Sons Inc.
      elastic fibres, fibrillin, fibrillinopathies, microfibrils

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          Summary

          Fibrillin microfibrils are indispensable structural elements of connective tissues in multicellular organisms from early metazoans to humans. They have an extensible periodic beaded organization, and support dynamic tissues such as ciliary zonules that suspend the lens. In tissues that express elastin, including blood vessels, skin and lungs, microfibrils support elastin deposition and shape the functional architecture of elastic fibres. The vital contribution of microfibrils to tissue form and function is underscored by the heritable fibrillinopathies, especially Marfan syndrome with severe elastic, ocular and skeletal tissue defects. Research since the early 1990s has advanced our knowledge of biology of microfibrils, yet understanding of their mechanical and homeostatic contributions to tissues remains far from complete. This review is a personal reflection on key insights, and puts forward the conceptual hypothesis that microfibrils are structural ‘tensometers’ that direct cells to monitor and respond to altered tissue mechanics.

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          Asprosin, a Fasting-Induced Glucogenic Protein Hormone.

          Hepatic glucose release into the circulation is vital for brain function and survival during periods of fasting and is modulated by an array of hormones that precisely regulate plasma glucose levels. We have identified a fasting-induced protein hormone that modulates hepatic glucose release. It is the C-terminal cleavage product of profibrillin, and we name it Asprosin. Asprosin is secreted by white adipose, circulates at nanomolar levels, and is recruited to the liver, where it activates the G protein-cAMP-PKA pathway, resulting in rapid glucose release into the circulation. Humans and mice with insulin resistance show pathologically elevated plasma asprosin, and its loss of function via immunologic or genetic means has a profound glucose- and insulin-lowering effect secondary to reduced hepatic glucose release. Asprosin represents a glucogenic protein hormone, and therapeutically targeting it may be beneficial in type II diabetes and metabolic syndrome.
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            Atenolol versus losartan in children and young adults with Marfan's syndrome.

            Aortic-root dissection is the leading cause of death in Marfan's syndrome. Studies suggest that with regard to slowing aortic-root enlargement, losartan may be more effective than beta-blockers, the current standard therapy in most centers.
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              Fibronectins, their fibrillogenesis, and in vivo functions.

              Fibronectin (FN) is a multidomain protein with the ability to bind simultaneously to cell surface receptors, collagen, proteoglycans, and other FN molecules. Many of these domains and interactions are also involved in the assembly of FN dimers into a multimeric fibrillar matrix. When, where, and how FN binds to its various partners must be controlled and coordinated during fibrillogenesis. Steps in the process of FN fibrillogenesis including FN self-association, receptor activities, and intracellular pathways have been under intense investigation for years. In this review, the domain organization of FN including the extra domains and variable region that are controlled by alternative splicing are described. We discuss how FN-FN and cell-FN interactions play essential roles in the initiation and progression of matrix assembly using complementary results from cell culture and embryonic model systems that have enhanced our understanding of this process.
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                Author and article information

                Contributors
                cay.kielty@manchester.ac.uk
                Journal
                Int J Exp Pathol
                Int J Exp Pathol
                10.1111/(ISSN)1365-2613
                IEP
                International Journal of Experimental Pathology
                John Wiley and Sons Inc. (Hoboken )
                0959-9673
                1365-2613
                14 September 2017
                August 2017
                14 September 2017
                : 98
                : 4 ( doiID: 10.1111/iep.2017.98.issue-4 )
                : 172-190
                Affiliations
                [ 1 ] Wellcome Trust Centre for Cell‐Matrix Research School of Biological Sciences Faculty of Biology, Medicine and Health University of Manchester Manchester UK
                Author notes
                [*] [* ] Correspondence:

                Cay M. Kielty

                Wellcome Trust Centre for Cell‐Matrix Research

                School of Biological Sciences

                Faculty of Biology, Medicine and Health

                University of Manchester

                Manchester M13 9PT

                UK

                Tel.: +0161 275 5072

                Fax: +161 275 5082

                E‐mail: cay.kielty@ 123456manchester.ac.uk

                Author information
                http://orcid.org/0000-0003-1610-6881
                Article
                IEP12239
                10.1111/iep.12239
                5639267
                28905442
                241c0441-2d1c-4167-be44-49d23f46c9b3
                © 2017 The Author. International Journal of Experimental Pathology published by John Wiley & Sons Ltd on behalf of Company of the International Journal of Experimental Pathology (CIJEP).

                This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                : 29 March 2017
                : 13 July 2017
                Page count
                Figures: 7, Tables: 0, Pages: 19, Words: 13407
                Funding
                Funded by: Medical Research Council
                Funded by: Biotechnology and Biological Sciences Research Council
                Funded by: Wellcome Trust
                Award ID: 203128/Z/16/Z
                Funded by: British Heart Foundation
                Funded by: Arthritis Research UK
                Funded by: Royal Society
                Categories
                Review Article
                Review Article
                Custom metadata
                2.0
                iep12239
                August 2017
                Converter:WILEY_ML3GV2_TO_NLMPMC version:5.2.1 mode:remove_FC converted:13.10.2017

                Pathology
                elastic fibres,fibrillin,fibrillinopathies,microfibrils
                Pathology
                elastic fibres, fibrillin, fibrillinopathies, microfibrils

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