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

Dimers of mitochondrial ATP synthase form the permeability transition pore

(2013) V. Giorgio et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Quantification of birefringence readily measures the level of muscle damage in zebrafish

(2012) Joachim Berger et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Autophagy induction rescues muscular dystrophy

(2011) Paolo Grumati et al.   Autophagy

Physical exercise stimulates autophagy in normal skeletal muscles but is detrimental for collagen VI-deficient muscles

(2011) Paolo Grumati et al.   Autophagy

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

(2011) P. Sabatelli et al.   JOURNAL OF CELLULAR PHYSIOLOGY

During autophagy mitochondria elongate, are spared from degradation and sustain cell viability

(2011) Ligia C. Gomes et al.   NATURE CELL BIOLOGY

Cyclosporine A in Ullrich Congenital Muscular Dystrophy: Long-Term Results

(2011) Luciano Merlini et al.   Oxidative Medicine and Cellular Longevity

Inhibition of Drp1-dependent mitochondrial fragmentation and apoptosis by a polypeptide antagonist of calcineurin

(2010) G M Cereghetti et al.   CELL DEATH AND DIFFERENTIATION

Zebrafish models of collagen VI-related myopathies

(2010) W.R. Telfer et al.   HUMAN MOLECULAR GENETICS

The Functional Role of Calcineurin in Hypertrophy, Regeneration, and Disorders of Skeletal Muscle

(2010) Kunihiro Sakuma et al.   JOURNAL OF BIOMEDICINE AND BIOTECHNOLOGY

Autophagy is defective in collagen VI muscular dystrophies and its reactivation rescues myofiber degeneration

(2010) Paolo Grumati et al.   NATURE MEDICINE

Isoform-Specific Inhibition of Cyclophilins

(2009) Sebastian Daum et al.   BIOCHEMISTRY

The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects inCol6a1−/−myopathic mice

(2009) T Tiepolo et al.   BRITISH JOURNAL OF PHARMACOLOGY

Inducible activation of Akt increases skeletal muscle mass and force without satellite cell activation

(2009) Bert Blaauw et al.   FASEB JOURNAL

Genetic ablation of cyclophilin D rescues mitochondrial defects and prevents muscle apoptosis in collagen VI myopathic mice

(2009) Elena Palma et al.   HUMAN MOLECULAR GENETICS

Six1a is required for the onset of fast muscle differentiation in zebrafish

(2008) Dmitri A. Bessarab et al.   DEVELOPMENTAL BIOLOGY

Exon skipping mutations in collagen VI are common and are predictive for severity and inheritance

(2008) A.K. Lampe et al.   HUMAN MUTATION

Autosomal recessive myosclerosis myopathy is a collagen VI disorder

(2008) L. Merlini et al.   NEUROLOGY

Therapy of collagen VI-related myopathies (Bethlem and Ullrich)

(2008) Luciano Merlini et al.   Neurotherapeutics

Dephosphorylation by calcineurin regulates translocation of Drp1 to mitochondria

(2008) G. M. Cereghetti et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies

(2008) Luciano Merlini et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA