[HTML][HTML] The polyphenol pterostilbene ameliorates the myopathic phenotype of collagen VI deficient mice via autophagy induction

S Metti, L Gambarotto, M Chrisam, ML Spina… - Frontiers in Cell and …, 2020 - frontiersin.org
The induction of autophagy, the catabolic pathway by which damaged or unnecessary
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

M Chrisam, M Pirozzi, S Castagnaro, B Blaauw… - Autophagy, 2015 - Taylor & Francis
Autophagy is a self-degradative process responsible for the clearance of damaged or
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 …

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 …

[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 …

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 …

Autophagy induction rescues muscular dystrophy

P Grumati, L Coletto, M Sandri, P Bonaldo - Autophagy, 2011 - Taylor & Francis
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 …

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 …

[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 …

[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 …