Melanocytes from Patients Affected by Ullrich Congenital Muscular Dystrophy and Bethlem Myopathy have Dysfunctional Mitochondria That Can be Rescued with Cyclophilin Inhibitors (Q34547398)

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English	Melanocytes from Patients Affected by Ullrich Congenital Muscular Dystrophy and Bethlem Myopathy have Dysfunctional Mitochondria That Can be Rescued with Cyclophilin Inhibitors	scientific article

Statements

scholarly article

1 reference

Europe PubMed Central

2 August 2017

Melanocytes from Patients Affected by Ullrich Congenital Muscular Dystrophy and Bethlem Myopathy have Dysfunctional Mitochondria That Can be Rescued with Cyclophilin Inhibitors (English)

1 reference

Europe PubMed Central

2 August 2017

congenital disorder

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Ullrich congenital muscular dystrophy

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1 reference

based on heuristic

inferred from title

Bethlem myopathy

1 reference

based on heuristic

inferred from title

Francesca Tagliavini

object named as

Francesca Tagliavini

2

0 references

Camilla Pellegrini

object named as

Camilla Pellegrini

4

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object named as

Nicoletta Zini

6

0 references

object named as

Spartaco Santi

8

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Valeria Petronilli

object named as

Valeria Petronilli

11

0 references

object named as

Paolo Bernardi

12

0 references

Luciano Merlini

object named as

Luciano Merlini

10

0 references

Patrizia Sabatelli

object named as

Patrizia Sabatelli

13

0 references

Alessandra Zulian

1

object named as

Alessandra Zulian

1 reference

Europe PubMed Central

2 August 2017

author name string

Erika Rizzo

3

1 reference

Europe PubMed Central

2 August 2017

Francesca Sardone

5

1 reference

Europe PubMed Central

2 August 2017

Nadir Mario Maraldi

7

1 reference

Europe PubMed Central

2 August 2017

Cesare Faldini

9

1 reference

Europe PubMed Central

2 August 2017

publication date

20 November 2014

1 reference

Europe PubMed Central

2 August 2017

Frontiers in Aging Neuroscience

1 reference

Europe PubMed Central

2 August 2017

6

1 reference

Europe PubMed Central

2 August 2017

324

1 reference

Europe PubMed Central

2 August 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Defective collagen VI α6 chain expression in the skeletal muscle of patients with collagen VI-related myopathies

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

The contribution of melanocytes to pathological scar formation during wound healing

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

The mitochondrial permeability transition pore: a mystery solved?

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Dimers of mitochondrial ATP synthase form the permeability transition pore

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Melanocytes--a novel tool to study mitochondrial dysfunction in Duchenne muscular dystrophy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Rapid detection of an ABT-737-sensitive primed for death state in cells using microplate-based respirometry

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Expression of collagen VI α5 and α6 chains in human muscle and in Duchenne muscular dystrophy-related muscle fibrosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Integrins: versatile receptors controlling melanocyte adhesion, migration and proliferation

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Functional and clinical relevance of chondroitin sulfate proteoglycan 4.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Debio-025 is more effective than prednisone in reducing muscular pathology in mdx mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Zebrafish models of collagen VI-related myopathies

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Isoform-specific inhibition of cyclophilins

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Multiparameter metabolic analysis reveals a close link between attenuated mitochondrial bioenergetic function and enhanced glycolysis dependency in human tumor cells

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Collagen VI related muscle disorders

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Dystroglycan in skin and cutaneous cells: beta-subunit is shed from the cell surface

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Collagen type VI and related disorders: Bethlem myopathy and Ullrich scleroatonic muscular dystrophy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Biglycan organizes collagen VI into hexagonal-like networks resembling tissue structures

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

The mitochondrial permeability transition, release of cytochrome c and cell death. Correlation with the duration of pore openings in situ

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

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.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Mitochondria and cell death. Mechanistic aspects and methodological issues

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Bethlem myopathy: a slowly progressive congenital muscular dystrophy with contractures

2 references

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Imaging the permeability pore transition in single mitochondria

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

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

1 reference

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7 July 2018

Integrin and Arg-Gly-Asp dependence of cell adhesion to the native and unfolded triple helix of collagen type VI

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4238408

7 July 2018

Calcium and pyridine nucleotide interaction in mitochondrial membranes

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7 July 2018

Benign myopathy, with autosomal dominant inheritance. A report on three pedigrees

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7 July 2018

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

1 reference

https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

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

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https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

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

1 reference

https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

Oxidative stress by monoamine oxidases is causally involved in myofiber damage in muscular dystrophy

1 reference

https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

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

1 reference

https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

Arachidonic acid released by phospholipase A(2) activation triggers Ca(2+)-dependent apoptosis through the mitochondrial pathway

1 reference

https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

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

1 reference

https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

Melanocytes: from morphology to application.

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https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

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

1 reference

https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

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

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https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

Autosomal recessive myosclerosis myopathy is a collagen VI disorder

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21 January 2018

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

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21 January 2018

Expression of the collagen VI α5 and α6 chains in normal human skin and in skin of patients with collagen VI-related myopathies

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27 October 2018

https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

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

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21 January 2018

Bethlem myopathy: Early-onset benign autosomal dominant myopathy with contractures. Description of two new families

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https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

Effects on Collagen VI mRNA Stability and Microfibrillar Assembly of ThreeCOL6A2Mutations in Two Families with Ullrich Congenital Muscular Dystrophy

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21 January 2018

Diminished binding of mutated collagen VI to the extracellular matrix surrounding myocytes

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https://api.crossref.org/works/10.3389%2FFNAGI.2014.00324

21 January 2018

Diagnosis of merosin (laminin-2) deficient congenital muscular dystrophy by skin biopsy

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https://pubmed.ncbi.nlm.nih.gov/25477819

12 December 2020

based on heuristic

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Biosynthesis of chick type VI collagen. II. Processing and secretion in fibroblasts and smooth muscle cells

1 reference

https://pubmed.ncbi.nlm.nih.gov/25477819

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expression of integrin receptors and their role in adhesion, spreading and migration of normal human melanocytes

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https://pubmed.ncbi.nlm.nih.gov/25477819

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based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.3389/FNAGI.2014.00324

1 reference

Europe PubMed Central

2 August 2017

1 reference

Europe PubMed Central

2 August 2017

1 reference

Europe PubMed Central

2 August 2017

ResearchGate publication ID

0 references

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