Basement membrane collagens and disease mechanisms

Essays in Biochemistry
Anna GatsevaTom Van Agtmael

Abstract

Basement membranes (BMs) are specialised extracellular matrix (ECM) structures and collagens are a key component required for BM function. While collagen IV is the major BM collagen, collagens VI, VII, XV, XVII and XVIII are also present. Mutations in these collagens cause rare multi-systemic diseases but these collagens have also been associated with major common diseases including stroke. Developing treatments for these conditions will require a collective effort to increase our fundamental understanding of the biology of these collagens and the mechanisms by which mutations therein cause disease. Novel insights into pathomolecular disease mechanisms and cellular responses to these mutations has been exploited to develop proof-of-concept treatment strategies in animal models. Combined, these studies have also highlighted the complexity of the disease mechanisms and the need to obtain a more complete understanding of these mechanisms. The identification of pathomolecular mechanisms of collagen mutations shared between different disorders represent an attractive prospect for treatments that may be effective across phenotypically distinct disorders.

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Citations

Sep 27, 2019·International Journal of Molecular Sciences·Ha Thi Thu NguyenToshitaka Oohashi
Apr 23, 2020·Journal of Cell Science·José C Pastor-Pareja
Mar 19, 2021·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·Alexandra PokhilkoM Zameel Cader
Apr 22, 2021·Seminars in Cell & Developmental Biology·Tiao BuC Yan Cheng
May 11, 2021·Frontiers in Oncology·Yunchun ZhaoCaihong Zheng
May 27, 2021·Expert Opinion on Drug Discovery·Marialucia Gallorini, Simone Carradori
Aug 13, 2021·Bioscience Reports·Erin BolandTom Van Agtmael

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Methods Mentioned

BETA
protein folding
antisense oligonucleotide
antisense oligonucleotides

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