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Mitochondrial dysfunction and defective autophagy in the pathogenesis of collagen VI muscular dystrophies

Cold Spring Harbor perspectives in biology, 2013-05, Vol.5 (5), p.a011387-a011387

2013

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The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects in Col6a1−/− myopathic mice

British journal of pharmacology, 2009-07, Vol.157 (6), p.1045-1052

2009

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NIM811, a cyclophilin inhibitor without immunosuppressive activity, is beneficial in collagen VI congenital muscular dystrophy models

Human molecular genetics, 2014-10, Vol.23 (20), p.5353-5363

2014

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Autosomal recessive myosclerosis myopathy is a collagen VI disorder

Neurology, 2008-10, Vol.71 (16), p.1245

2008

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Monoamine oxidase inhibition prevents mitochondrial dysfunction and apoptosis in myoblasts from patients with collagen VI myopathies

Free radical biology & medicine, 2014-10, Vol.75, p.40-47

2014

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Consensus-Based Single-Phase Control of Three-Phase Inverter for Current Sharing in Three-Phase Four-Wire Isolated AC Microgrids

IEEE access, 2022, Vol.10, p.1-1

2022

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CPT-Based Multi-Objective Strategy for Power Quality Enhancement in Three-Phase Three-Wire Systems Under Distorted and Unbalanced Voltage Conditions

IEEE access, 2021, Vol.9, p.53078-53095

2021

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First steps to define murine amniotic fluid stem cell microenvironment

Scientific reports, 2016-11, Vol.6 (1), p.37080-37080, Article 37080

2016

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Enhanced Control Strategy Based on Virtual Impedance Loops to Achieve the Sharing of Imbalance and Harmonic in 3-Phase 4-Wire Isolated Microgrids

IEEE transactions on power delivery, 2022-08, Vol.37 (4), p.3412-3415

2022

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Autosomal recessive Bethlem myopathy

Neurology, 2009-12, Vol.73 (22), p.1883

2009

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A Graphical Method to Assess the Technical Feasibility of Intentional Islanding of Distributed Synchronous Generators

IEEE transactions on power delivery, 2023-02, Vol.38 (1), p.742-745

2023

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IEEE_Xplore

Dystrophin restoration in skeletal, heart and skin arrector pili smooth muscle of mdx mice by ZM2 NP-AON complexes

Gene therapy, 2010-03, Vol.17 (3), p.432-438

2010

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Critical evaluation of the use of cell cultures for inclusion in clinical trials of patients affected by collagen VI myopathies

Journal of cellular physiology, 2012-07, Vol.227 (7), p.2927-2935

2012

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Transcriptomics analysis in collagen VI myopathy: Role of circadian genes using novel fluidic card tools

Neuromuscular disorders : NMD, 2016-10, Vol.26, p.S90-S91

2016

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P5 Detection of circulating miRNAs in serum in a mouse model of Collagen VI Deficiency

Neuromuscular disorders : NMD, 2014-03, Vol.24, p.S8-S8

2014

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P.1.8 Genetic ablation of p66Shc rescues functional and morphological abnormalities in collagen VI dystrophic mice

Neuromuscular disorders : NMD, 2013-10, Vol.23 (9), p.744-744

2013

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G.P.15 Whole transcriptome expression profiling in COL6a1 null mice shows deregulation of circadian clock genes as exploratory COL6 myopathies biomarkers

Neuromuscular disorders : NMD, 2012-10, Vol.22 (9), p.826-827

2012

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T.P.22 Nanoparticles as delivery systems for antisense oligoribonucleotides: Biodistribution studies and definition of the release kinetic in treated mdx mice

Neuromuscular disorders : NMD, 2012-10, Vol.22 (9), p.859-859

2012

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P2.3 Monoamine oxidase inhibitors reduce mitochondrial ROS accumulation and dysfunction in patients with collagen VI myopathies

Neuromuscular disorders : NMD, 2011-10, Vol.21 (9), p.661-661

2011

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EM.I.2 Toward a mitochondrial therapy of collagen VI muscular dystrophies

Neuromuscular disorders : NMD, 2009-09, Vol.19 (8), p.598-598

2009

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O.17 Autophagy thwarts collagen VI muscular dystrophies

Neuromuscular disorders : NMD, 2011-10, Vol.21 (9), p.749-749

2011

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P4.50 Mitochondrial therapy with Cyclosporine A in patients with Ullrich Congenital Muscular Dystrophy

Neuromuscular disorders : NMD, 2011-10, Vol.21 (9), p.719-719

2011

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Muscle ring finger-1 is required to prevent age-related cardiac hypertrophy and interstitial remodelling

European heart journal, 2020-11, Vol.41 (Supplement_2)

2020

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Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency

Nature genetics, 2003-12, Vol.35 (4), p.367-371

2003

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H-1 Collagen VI myopathies: pathogenic mechanisms and novel therapeutic perspectives

Acta myologica, 2011-10, Vol.30 (2), p.149-149

Bonaldo, P.

2011

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Mitochondrial dysfunction and defective autophagy in the pathogenesis of collagen VI muscular dystrophies

The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects in Col6a1−/− myopathic mice

NIM811, a cyclophilin inhibitor without immunosuppressive activity, is beneficial in collagen VI congenital muscular dystrophy models

Autosomal recessive myosclerosis myopathy is a collagen VI disorder

Monoamine oxidase inhibition prevents mitochondrial dysfunction and apoptosis in myoblasts from patients with collagen VI myopathies

Consensus-Based Single-Phase Control of Three-Phase Inverter for Current Sharing in Three-Phase Four-Wire Isolated AC Microgrids

CPT-Based Multi-Objective Strategy for Power Quality Enhancement in Three-Phase Three-Wire Systems Under Distorted and Unbalanced Voltage Conditions

First steps to define murine amniotic fluid stem cell microenvironment

Enhanced Control Strategy Based on Virtual Impedance Loops to Achieve the Sharing of Imbalance and Harmonic in 3-Phase 4-Wire Isolated Microgrids

Autosomal recessive Bethlem myopathy

A Graphical Method to Assess the Technical Feasibility of Intentional Islanding of Distributed Synchronous Generators

Dystrophin restoration in skeletal, heart and skin arrector pili smooth muscle of mdx mice by ZM2 NP-AON complexes

Critical evaluation of the use of cell cultures for inclusion in clinical trials of patients affected by collagen VI myopathies

Transcriptomics analysis in collagen VI myopathy: Role of circadian genes using novel fluidic card tools

P5 Detection of circulating miRNAs in serum in a mouse model of Collagen VI Deficiency

P.1.8 Genetic ablation of p66Shc rescues functional and morphological abnormalities in collagen VI dystrophic mice

G.P.15 Whole transcriptome expression profiling in COL6a1 null mice shows deregulation of circadian clock genes as exploratory COL6 myopathies biomarkers

T.P.22 Nanoparticles as delivery systems for antisense oligoribonucleotides: Biodistribution studies and definition of the release kinetic in treated mdx mice

P2.3 Monoamine oxidase inhibitors reduce mitochondrial ROS accumulation and dysfunction in patients with collagen VI myopathies

EM.I.2 Toward a mitochondrial therapy of collagen VI muscular dystrophies

O.17 Autophagy thwarts collagen VI muscular dystrophies

P4.50 Mitochondrial therapy with Cyclosporine A in patients with Ullrich Congenital Muscular Dystrophy

Muscle ring finger-1 is required to prevent age-related cardiac hypertrophy and interstitial remodelling

Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency

H-1 Collagen VI myopathies: pathogenic mechanisms and novel therapeutic perspectives

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