Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies (Q24647603)

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Paolo Bernardi

object named as

Paolo Bernardi

10

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author name string

Alessia Angelin

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Tania Tiepolo

3

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Patrizia Sabatelli

5

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Alessandra Zamparelli

6

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Alessandra Ferlini

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Nadir M Maraldi

8

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Paolo Bonaldo

Proceedings of the National Academy of Sciences of the United States of America

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language of work or name

English

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publication date

1 April 2008

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published in

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volume

105

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issue

13

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page(s)

5225-9

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cites work

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Mutations in COL6A3 cause severe and mild phenotypes of Ullrich congenital muscular dystrophy

7 April 2017

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Ullrich scleroatonic muscular dystrophy is caused by recessive mutations in collagen type VI

7 April 2017

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The Ca2+-induced membrane transition in mitochondria. I. The protective mechanisms

7 April 2017

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A mitochondrial paradigm of metabolic and degenerative diseases, aging, and cancer: a dawn for evolutionary medicine

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

7 April 2017

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The nonimmunosuppressive cyclosporin analogs NIM811 and UNIL025 display nanomolar potencies on permeability transition in brain-derived mitochondria.

27 September 2017

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New molecular mechanism for Ullrich congenital muscular dystrophy: a heterozygous in-frame deletion in the COL6A1 gene causes a severe phenotype

27 September 2017

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Muscular dystrophy--reason for optimism?

27 September 2017

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Opening of the mitochondrial permeability transition pore causes depletion of mitochondrial and cytosolic NAD+ and is a causative event in the death of myocytes in postischemic reperfusion of the heart.

27 September 2017

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Mitochondrial membrane potential and neuronal glutamate excitotoxicity: mortality and millivolts

27 September 2017

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Fluctuations in mitochondrial membrane potential caused by repetitive gating of the permeability transition pore

27 September 2017

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Mitochondria and cell death. Mechanistic aspects and methodological issues

27 September 2017

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Transient and long-lasting openings of the mitochondrial permeability transition pore can be monitored directly in intact cells by changes in mitochondrial calcein fluorescence

27 September 2017

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Imaging the permeability pore transition in single mitochondria

27 September 2017

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Mitochondrial calcium overload: A general mechanism for cell-necrosis in muscle diseases

27 September 2017

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Mitochondria Are Excitable Organelles Capable of Generating and Conveying Electrical and Calcium Signals

27 September 2017

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Calcium and pyridine nucleotide interaction in mitochondrial membranes

30 May 2018

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

30 May 2018

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Arachidonic acid causes cell death through the mitochondrial permeability transition. Implications for tumor necrosis factor-alpha aopototic signaling

21 January 2018

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Desensitization of the permeability transition pore by cyclosporin a prevents activation of the mitochondrial apoptotic pathway and liver damage by tumor necrosis factor-alpha

21 January 2018

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Kongenitale, atonisch-sklerotische Muskeldystrophie, ein weiterer Typus der heredodegenerativen Erkrankungen des neuromuskulären Systems

21 January 2018

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

21 January 2018

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X-ray structure of a decameric cyclophilin-cyclosporin crystal complex

21 January 2018

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Bethlem myopathy (BETHLEM) and Ullrich scleroatonic muscular dystrophy: 100th ENMC international workshop, 23-24 November 2001, Naarden, The Netherlands

21 January 2018

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Dominant collagen VI mutations are a common cause of Ullrich congenital muscular dystrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.0800962105

Novel COL6A1 splicing mutation in a family affected by mild Bethlem myopathy

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.0800962105

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

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2278179

Quantitative assay by flow cytometry of the mitochondrial membrane potential in intact cells

28 November 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/18362356

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1073/PNAS.0800962105

1 reference

ADS bibcode

2008PNAS..105.5225M

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1 reference

1 reference

PubMed publication ID

18362356

1 reference