NDLI: Matrix remodeling in systemic sclerosis

Content Provider	Springer Nature Link
Author	Leask, Andrew
Copyright Year	2015
Abstract	Systemic sclerosis (SSc, scleroderma) is an often-fatal disease characterized by connective tissue fibrosis of skin and internal organs. In scleroderma, there is an excessive production and accumulation of extracellular matrix (ECM) components resulting from an increase in collagen synthesis and matrix stability. Understanding how this how excessive ECM is produced and remodeled may represent a novel therapeutic approach. In this review, the transcription factors and collagen-modifying enzymes underlying collagen overexpression and enhancing stability in SSc are discussed. Moreover, the role of matrix stiffness in promoting fibrosis via a feed-forward mechanism is discussed. Indeed, the emerging evidence is that enhanced ECM remodeling resulting in increased ECM stiffness may be sufficient in itself to sustain persistence fibrosis in SSc.
Starting Page	559
Ending Page	563
Page Count	5
File Format	PDF
ISSN	18632297
Journal	Springer Seminars in Immunopathology
Volume Number	37
Issue Number	5
e-ISSN	18632300
Language	English
Publisher	Springer Berlin Heidelberg
Publisher Date	2015-07-04
Publisher Place	Berlin, Heidelberg
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Immunology Internal Medicine
Content Type	Text
Resource Type	Article
Subject	Immunology and Allergy Immunology

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1	Ministry of Education (GoI), Department of Higher Education	Sanctioning Authority	https://www.education.gov.in/ict-initiatives
2	Indian Institute of Technology Kharagpur	Host Institute of the Project: The host institute of the project is responsible for providing infrastructure support and hosting the project	https://www.iitkgp.ac.in
3	National Digital Library of India Office, Indian Institute of Technology Kharagpur	The administrative and infrastructural headquarters of the project	Dr. B. Sutradhar bsutra@ndl.gov.in
4	Project PI / Joint PI	Principal Investigator and Joint Principal Investigators of the project	Dr. B. Sutradhar bsutra@ndl.gov.in Prof. Saswat Chakrabarti will be added soon
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Matrix remodeling in systemic sclerosis