(Q46085120)

19 December 2017

title

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

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19 December 2017

author

Paolo Bernardi

8

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19 December 2017

Emy Basso

6

object named as

Emy Basso

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19 December 2017

author name string

Elena Palma

1

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19 December 2017

Tania Tiepolo

2

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19 December 2017

Alessia Angelin

3

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19 December 2017

Patrizia Sabatelli

4

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19 December 2017

Nadir M Maraldi

5

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19 December 2017

Michael A Forte

7

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19 December 2017

Paolo Bonaldo

9

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19 December 2017

publication date

17 March 2009

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19 December 2017

published in

Human Molecular Genetics

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19 December 2017

volume

18

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19 December 2017

issue

11

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19 December 2017

page(s)

2024-2031

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19 December 2017

Collagen VI related muscle disorders

cites work

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Dysfunction of mitochondria and sarcoplasmic reticulum in the pathogenesis of collagen VI muscular dystrophies

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Type VI collagen anchors endothelial basement membranes by interacting with type IV collagen

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Muscle interstitial fibroblasts are the main source of collagen VI synthesis in skeletal muscle: implications for congenital muscular dystrophy types Ullrich and Bethlem

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Autosomal recessive myosclerosis myopathy is a collagen VI disorder

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Three novel collagen VI chains with high homology to the alpha3 chain

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Collagen VI deficiency induces early onset myopathy in the mouse: an animal model for Bethlem myopathy.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

The mitochondrial permeability transition from in vitro artifact to disease target

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Interactions of cyclophilin with the mitochondrial inner membrane and regulation of the permeability transition pore, and cyclosporin A-sensitive channel.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Mitochondrial dysfunction in the pathogenesis of Ullrich congenital muscular dystrophy and prospective therapy with cyclosporins

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Inhibition of Ca2(+)-induced large-amplitude swelling of liver and heart mitochondria by cyclosporin is probably caused by the inhibitor binding to mitochondrial-matrix peptidyl-prolyl cis-trans isomerase and preventing it interacting with the adeni

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Loss of cyclophilin D reveals a critical role for mitochondrial permeability transition in cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Properties of the permeability transition pore in mitochondria devoid of Cyclophilin D.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Cyclophilin D-dependent mitochondrial permeability transition regulates some necrotic but not apoptotic cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Cyclophilin D is a component of mitochondrial permeability transition and mediates neuronal cell death after focal cerebral ischemia

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Altered skeletal muscle phenotypes in calcineurin Aalpha and Abeta gene-targeted mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Dephosphorylation by calcineurin regulates translocation of Drp1 to mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Inhibition of human immunodeficiency virus type 1 replication in human cells by Debio-025, a novel cyclophilin binding agent

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Cyclophilin and peptidyl-prolyl cis-trans isomerase are probably identical proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Cyclophilin A is a proinflammatory cytokine that activates endothelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Cyclophilin A as a novel biphasic mediator of endothelial activation and dysfunction.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Cyclophilin A may contribute to the inflammatory processes in rheumatoid arthritis through induction of matrix degrading enzymes and inflammatory cytokines from macrophages

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Extracellular cyclophilins contribute to the regulation of inflammatory responses

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Preferential chemotaxis of activated human CD4+ T cells by extracellular cyclophilin A

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Cyclophilin C-associated protein is up-regulated during wound healing

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Cyclophilin, TRIM5, and innate immunity to HIV-1

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Cyclophilin inhibitors in hepatitis C viral infection

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Peptidyl-prolyl cis-trans isomerases (immunophilins) and their roles in parasite biochemistry, host-parasite interaction and antiparasitic drug action

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Roles of cyclophilins in cancers and other organ systems

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Phosphate is essential for inhibition of the mitochondrial permeability transition pore by cyclosporin A and by cyclophilin D ablation

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Bupivacaine myotoxicity is mediated by mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

Primary mouse myoblast purification, characterization, and transplantation for cell-mediated gene therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDP126

21 January 2018

Identifiers

DOI

10.1093/HMG/DDP126

1 reference

19 December 2017

PubMed ID

19293339

1 reference

19 December 2017