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Article
Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration
Autophagy is often believed to be elevated in disease, contributing to pathogenesis. Paolo Bonaldo and his colleagues now show that it is actually too little autophagy that occurs in some forms of muscular dys...
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Article
Collagen VI regulates peripheral nerve regeneration by modulating macrophage recruitment and polarization
Macrophages contribute to peripheral nerve regeneration and produce collagen VI, an extracellular matrix protein involved in nerve function. Here, we show that collagen VI is critical for macrophage migration ...
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Article
The Role of Collagens in Peripheral Nerve Myelination and Function
In the peripheral nervous system, myelin is formed by Schwann cells, which are surrounded by a basal lamina. Extracellular matrix (ECM) molecules in the basal lamina play an important role in regulating Schwan...
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Article
Collagen VI is required for the structural and functional integrity of the neuromuscular junction
The synaptic cleft of the neuromuscular junction (NMJ) consists of a highly specialized extracellular matrix (ECM) involved in synapse maturation, in the juxtaposition of pre- to post-synaptic areas, and in en...
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Article
Role of adiponectin in the metabolism of skeletal muscles in collagen VI–related myopathies
The role of adiponectin has been particularly deepened in diabetic muscles while the study of adiponectin in hereditary myopathies has been marginally investigated. Here, we report the study about adiponectin ...
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Article
Open AccessBicalutamide and Trehalose Ameliorate Spinal and Bulbar Muscular Atrophy Pathology in Mice
Spinal and bulbar muscular atrophy (SBMA) is characterized by motor neuron (MN) degeneration that leads to slowly progressive muscle weakness. It is considered a neuromuscular disease since muscle has a primar...
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Article
Open AccessCollagen VI sustains cell stemness and chemotherapy resistance in glioblastoma
Microenvironmental factors are known fundamental regulators of the phenotype and aggressiveness of glioblastoma (GBM), the most lethal brain tumor, characterized by fast progression and marked resistance to tr...
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Article
Open AccessGBA1 inactivation in oligodendrocytes affects myelination and induces neurodegenerative hallmarks and lipid dyshomeostasis in mice
Mutations in the β-glucocerebrosidase (GBA1) gene do cause the lysosomal storage Gaucher disease (GD) and are among the most frequent genetic risk factors for Parkinson’s disease (PD). So far, studies on both neu...