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

Dysfunction of Mitochondria and Sarcoplasmic Reticulum in the Pathogenesis of Collagen VI Muscular Dystrophies

(2010) Paolo Bernardi et al.   Annals of the New York Academy of Sciences

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

Muscle Interstitial Fibroblasts Are the Main Source of Collagen VI Synthesis in Skeletal Muscle: Implications for Congenital Muscular Dystrophy Types Ullrich and Bethlem

(2009) Yaqun Zou et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Inhibition of Human Immunodeficiency Virus Type 1 Replication in Human Cells by Debio-025, a Novel Cyclophilin Binding Agent

(2008) R. G. Ptak et al.   ANTIMICROBIAL AGENTS AND CHEMOTHERAPY

Altered threshold of the mitochondrial permeability transition pore in Ullrich congenital muscular dystrophy

(2008) Alessia Angelin et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Recent progress in elucidating the molecular mechanism of the mitochondrial permeability transition pore

(2008) Anna W.C. Leung et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Cyclosporine A treatment for Ullrich congenital muscular dystrophy: a cellular study of mitochondrial dysfunction and its rescue

(2008) D. Hicks et al.   BRAIN

Investigation of Debio 025, a cyclophilin inhibitor, in the dystrophic mdx mouse, a model for Duchenne muscular dystrophy

(2008) J Reutenauer et al.   BRITISH JOURNAL OF PHARMACOLOGY

An enhancer required for transcription of the Col6a1 gene in muscle connective tissue is induced by signals released from muscle cells

(2008) Paola Braghetta et al.   EXPERIMENTAL CELL RESEARCH

Three Novel Collagen VI Chains with High Homology to the α3 Chain

(2008) Sudheer Kumar Gara et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Phosphate Is Essential for Inhibition of the Mitochondrial Permeability Transition Pore by Cyclosporin A and by Cyclophilin D Ablation

(2008) Emy Basso et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Genetic and pharmacologic inhibition of mitochondrial-dependent necrosis attenuates muscular dystrophy

(2008) Douglas P Millay et al.   NATURE MEDICINE

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