[HTML][HTML] The polyphenol pterostilbene ameliorates the myopathic phenotype of collagen VI deficient mice via autophagy induction
The induction of autophagy, the catabolic pathway by which damaged or unnecessary
cellular components are subjected to lysosome-mediated degradation and recycling, is …
cellular components are subjected to lysosome-mediated degradation and recycling, is …
Reactivation of autophagy by spermidine ameliorates the myopathic defects of collagen VI-null mice
Autophagy is a self-degradative process responsible for the clearance of damaged or
unnecessary cellular components. We have previously found that persistence of …
unnecessary cellular components. We have previously found that persistence of …
Sustained oral spermidine supplementation rescues functional and structural defects in COL6-deficient myopathic mice
L Gambarotto, S Metti, M Corpetti, M Baraldo… - Autophagy, 2023 - Taylor & Francis
ABSTRACT COL6 (collagen type VI)-related myopathies (COL6-RM) are a distinct group of
inherited muscle disorders caused by mutations of COL6 genes and characterized by early …
inherited muscle disorders caused by mutations of COL6 genes and characterized by early …
Physical exercise stimulates autophagy in normal skeletal muscles but is detrimental for collagen VI-deficient muscles
P Grumati, L Coletto, A Schiavinato, S Castagnaro… - Autophagy, 2011 - Taylor & Francis
Autophagy is a catabolic process that provides the degradation of altered/damaged
organelles through the fusion between autophagosomes and lysosomes. Proper regulation …
organelles through the fusion between autophagosomes and lysosomes. Proper regulation …
[HTML][HTML] Autophagy in mammalian development and differentiation
S Di Bartolomeo, L Latella, K Zarbalis… - Frontiers in Cell and …, 2021 - frontiersin.org
Autophagy is a cellular degradation and recycling process by which cytoplasmic
components, including macromolecules and organelles, are sequestered into specialized …
components, including macromolecules and organelles, are sequestered into specialized …
Activation of autophagy is required for muscle homeostasis during physical exercise
U Nair, DJ Klionsky - Autophagy, 2011 - Taylor & Francis
Skeletal muscle fibers of collagen VI null (Col6a1−/−) mice show signs of degeneration due
to a block in autophagy, leading to the accumulation of damaged mitochondria and …
to a block in autophagy, leading to the accumulation of damaged mitochondria and …
Autophagy induction rescues muscular dystrophy
Collagen VI is an extracellular matrix protein forming a microfibrillar network in the
endomysium of skeletal muscles. In humans, mutations in any of the three genes coding for …
endomysium of skeletal muscles. In humans, mutations in any of the three genes coding for …
Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration
P Grumati, L Coletto, P Sabatelli, M Cescon… - Nature medicine, 2010 - nature.com
Autophagy is crucial in the turnover of cell components, and clearance of damaged
organelles by the autophagic-lysosomal pathway is essential for tissue homeostasis …
organelles by the autophagic-lysosomal pathway is essential for tissue homeostasis …
[HTML][HTML] Extracellular collagen VI has prosurvival and autophagy instructive properties in mouse fibroblasts
S Castagnaro, M Chrisam, M Cescon… - Frontiers in …, 2018 - frontiersin.org
Collagen VI (ColVI) is an abundant and distinctive extracellular matrix protein secreted by
fibroblasts in different tissues. Human diseases linked to mutations on ColVI genes are …
fibroblasts in different tissues. Human diseases linked to mutations on ColVI genes are …
[HTML][HTML] Cyclosporin A Promotes in vivo Myogenic Response in Collagen VI-Deficient Myopathic Mice
F Gattazzo, S Molon, V Morbidoni… - Frontiers in Aging …, 2014 - frontiersin.org
Mutations of genes encoding for collagen VI cause various muscle diseases in humans,
including Bethlem myopathy and Ullrich congenital muscular dystrophy. Collagen VI null …
including Bethlem myopathy and Ullrich congenital muscular dystrophy. Collagen VI null …