Genetic disorders of the extracellular matrix

SR Lamandé, JF Bateman - The anatomical record, 2020 - Wiley Online Library
Mutations in the genes for extracellular matrix (ECM) components cause a wide range of
genetic connective tissues disorders throughout the body. The elucidation of mutations and …

Identifying collagen VI as a target of fibrotic diseases regulated by CREBBP/EP300

LM Williams, FE McCann, MA Cabrita… - Proceedings of the …, 2020 - National Acad Sciences
Fibrotic diseases remain a major cause of morbidity and mortality, yet there are few effective
therapies. The underlying pathology of all fibrotic conditions is the activity of myofibroblasts …

Genomic inbreeding trends, influential sire lines and selection in the global Thoroughbred horse population

BA McGivney, H Han, LR Corduff, LM Katz, T Tozaki… - Scientific Reports, 2020 - nature.com
The Thoroughbred horse is a highly valued domestic animal population under strong
selection for athletic phenotypes. Here we present a high resolution genomics-based …

Molecular phenotyping of white striping and wooden breast myopathies in chicken

C Praud, J Jimenez, E Pampouille… - Frontiers in …, 2020 - frontiersin.org
The White Striping (WS) and Wooden Breast (WB) defects are two myopathic syndromes
whose occurrence has recently increased in modern fast-growing broilers. The impact of …

Tendon extracellular matrix remodeling and defective cell polarization in the presence of collagen VI mutations

M Antoniel, F Traina, L Merlini, D Andrenacci, D Tigani… - Cells, 2020 - mdpi.com
Mutations in collagen VI genes cause two major clinical myopathies, Bethlem myopathy
(BM) and Ullrich congenital muscular dystrophy (UCMD), and the rarer myosclerosis …

Novel Mutations in COL6A3 That Associated With Peters' Anomaly Caused Abnormal Intracellular Protein Retention and Decreased Cellular Resistance to Oxidative …

Y Li, J Zhang, Y Dai, Y Fan, J Xu - Frontiers in Cell and …, 2020 - frontiersin.org
Peters' anomaly (PA) is a rare form of anterior segment dysgenesis characterized by central
corneal opacity accompanied by iridocorneal or lenticulo-corneal adhesions. Although …

Lower extremity muscle involvement in the intermediate and bethlem myopathy forms of COL6-related dystrophy and duchenne muscular dystrophy: A cross-sectional …

A Batra, DJ Lott, R Willcocks, SC Forbes… - Journal of …, 2020 - content.iospress.com
Collagen VI-related dystrophies (COL6-RDs) and Duchenne muscular dystrophy (DMD)
cause progressive muscle weakness and disability. COL6-RDs are caused by mutations in …

Matrixmetalloproteasen 1, 2, 3, 9, 13-und Kollagen Typ VI-mRNA-Expression humaner Chondrozyten in verschiedenen räumlichen Organisationsformen

A Egler - 2020 - ub01.uni-tuebingen.de
Die Arthrose (Osteoarthritis/OA) ist eine bis heute noch unvollständig verstandene
Erkrankung und es stehen demnach keine kausal oder präventiv wirkenden biologischen …