[HTML][HTML] Linking oxidative stress and DNA damage to changes in the expression of extracellular matrix components

SG Martins, R Zilhão, S Thorsteinsdóttir… - Frontiers in …, 2021 - frontiersin.org
Cells are subjected to endogenous [eg, reactive oxygen species (ROS), replication stress]
and exogenous insults (eg, UV light, ionizing radiation, and certain chemicals), which can …

[HTML][HTML] Immunobiology of inherited muscular dystrophies

JG Tidball, SS Welc… - Comprehensive …, 2018 - ncbi.nlm.nih.gov
The immune response to acute muscle damage is important for normal repair. However, in
chronic diseases such as many muscular dystrophies, the immune response can amplify …

The basement membrane as a structured surface–role in vascular health and disease

C Leclech, CF Natale, AI Barakat - Journal of cell science, 2020 - journals.biologists.com
The basement membrane (BM) is a thin specialized extracellular matrix that functions as a
cellular anchorage site, a physical barrier and a signaling hub. While the literature on the …

Misregulation of autophagy and protein degradation systems in myopathies and muscular dystrophies

M Sandri, L Coletto, P Grumati… - Journal of cell …, 2013 - journals.biologists.com
A number of recent studies have highlighted the importance of autophagy and the ubiquitin-
proteasome in the pathogenesis of muscle wasting in different types of inherited muscle …

[HTML][HTML] The development of the myotendinous junction. A review

B Charvet, F Ruggiero, D Le Guellec - Muscles, ligaments and …, 2012 - ncbi.nlm.nih.gov
The myotendinous junction (MTJ) is a complex specialized region located at the muscle-
tendon interface that represents the primary site of force transmission. Despite their different …

Laminin-211 in skeletal muscle function

J Holmberg, M Durbeej - Cell adhesion & migration, 2013 - Taylor & Francis
A chain is no stronger than its weakest link is an old idiom that holds true for muscle biology.
As the name implies, skeletal muscle's main function is to move the bones. However, for a …

Laminins and their receptors in the CNS

A Nirwane, Y Yao - Biological Reviews, 2019 - Wiley Online Library
Laminin, an extracellular matrix protein, is widely expressed in the central nervous system
(CNS). By interacting with integrin and non‐integrin receptors, laminin exerts a large variety …

Advances in CRISPR/Cas9 genome editing for the treatment of muscular dystrophies

S Fatehi, RM Marks, MJ Rok, L Perillat… - Human Gene …, 2023 - liebertpub.com
Muscular dystrophies (MDs) comprise a diverse group of inherited disorders characterized
by progressive muscle loss and weakness. Given the genetic etiology underlying MDs …

Autophagy is increased in laminin α2 chain-deficient muscle and its inhibition improves muscle morphology in a mouse model of MDC1A

V Carmignac, M Svensson, Z Körner… - Human molecular …, 2011 - academic.oup.com
Congenital muscular dystrophy caused by laminin α2 chain deficiency (also known as
MDC1A) is a severe and incapacitating disease, characterized by massive muscle wasting …

Linker proteins restore basement membrane and correct LAMA2-related muscular dystrophy in mice

JR Reinhard, S Lin, KK McKee, S Meinen… - Science translational …, 2017 - science.org
L AMA2-related muscular dystrophy (LAMA2 MD or MDC1A) is the most frequent form of
early-onset, fatal congenital muscular dystrophies. It is caused by mutations in LAMA2, the …