7 Result(s)
within
Matilde Cescon
Neurosciences
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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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Collagens and Muscle Diseases: A Focus on Collagen VI
The extracellular matrix is a three-dimensional network providing the proper microenvironment for muscle development and function. Different types of collagens play critical roles in skeletal muscle homeostasi...
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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 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...
