RESEARCH ARTICLE Mitochondrial Dysfunction Reveals the Role of mRNA Poly(A) Tail Regulation in Oculopharyngeal Muscular Dystrophy Pathogenesis

The Interplay of Mitophagy and Inflammation in Duchenne Muscular Dystrophy

Andrea L. Reid
Matthew S. Alexander

July 2021

Duchenne muscular dystrophy (DMD) is an X-linked neuromuscular disease caused by a pathogenic disrup...

A revised model for mitochondrial dysfunction in Duchenne muscular dystrophy

Ai Vu Hong
Mathilde Sanson
Isabelle Richard
David Israeli

September 2021

We recetly identified a signaling pathway that links the upregulation of miR-379 with a mitochondria...

Molecular model of OPMD.

Aymeric Chartier (713911)
Pierre Klein (713912)
Stéphanie Pierson (713913)
Nicolas Barbezier (713914)
Teresa Gidaro (688497)
François Casas (93372)
Steven Carberry (713915)
Paul Dowling (713916)
Laurie Maynadier (713917)
Maëlle Bellec (713918)
Martine Oloko (713919)
Claude Jardel (713920)
Bodo Moritz (146655)
George Dickson (123905)
Vincent Mouly (75137)
Kay Ohlendieck (1235)
Gillian Butler-Browne (75143)
Capucine Trollet (713921)
Martine Simonelig (713922)

March 2015

<p>The first molecular defect in OPMD would be a general decrease in the cleavage/polyadenylation re...

Mitochondrial Dysfunction Reveals the Role of mRNA Poly(A) Tail Regulation in Oculopharyngeal Muscular Dystrophy Pathogenesis

Chartier, Aymeric
Klein, Pierre
Pierson, Stéphanie
Barbezier, Nicolas
Gidaro, Teresa
Casas, François
Carberry, Steven
Dowling, Paul
Maynadier, Laurie
Bellec, Maëlle
Oloko, Martine
Jardel, Claude
Moritz, Bodo
Dickson, George
Mouly, Vincent
Ohlendieck, Kay
Butler-Browne, Gillian
Trollet, Capucine
Simonelig, Martine

March 2015

International audienceOculopharyngeal muscular dystrophy (OPMD), a late-onset disorder characterized...

Mitochondrial abnormalities in genetically assessed oculopharyngeal muscular dystrophy.

GAMBELLI S.
DE STEFANO R.
FRANCI M.
BRUTTINI F.
MALANDRINI, ALESSANDRO
GINANNESCHI, FEDERICA
BERTI, GIANNA
CARDAIOLI, ELENA
SALVADORI, CLAUDIO
MARI, FRANCESCA
ROSSI, ALESSANDRO
FEDERICO, ANTONIO
RENIERI, ALESSANDRA

January 2004

We report a family with a clinical diagnosis of oculopharyngeal muscular dystrophy in which muscle b...

Oculopharyngeal muscular dystrophy: Recent advances in the understanding of the molecular pathogenic mechanisms and treatment strategies

Abu-Baker, Aida
Rouleau, Guy A.

February 2007

AbstractOculopharyngeal muscular dystrophy (OPMD) is an adult-onset disorder characterized by progre...

RESEARCH ARTICLE Defects in Mitochondrial ATP Synthesis in Dystrophin-Deficient Mdx Skeletal Muscles May Be Caused by Complex I Insufficiency

Emma Rybalka
Cara A. Timpani
Matthew B. Cooke
Andrew D. Williams
Alan Hayes

August 2016

Duchenne Muscular Dystrophy is a chronic, progressive and ultimately fatal skeletal muscle wasting d...

Nuclear entrapment and extracellular depletion of PCOLCE is associated with muscle degeneration in oculopharyngeal muscular dystrophy

Raz, V.
Sterrenburg, E.
Routledge, S.
Venema, A.
Sluijs, B.M. van der
Trollet, C.
Dickson, G.
Engelen, B.G.M. van
Maarel, S.M. van der
Antoniou, M.N.

January 2013

Contains fulltext : 181501.pdf (publisher's version ) (Open Access)BACKGROUND: Mus...

Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients.

Anvar, Seyed, Yahya
T Hoen, Peter
Venema, Andrea
van Der Sluijs, Barbara
van Engelen, Baziel
Snoeck, Marc
Vissing, John
Trollet, Capucine
Dickson, George
Chartier, Aymeric
Simonelig, Martine
van Ommen, Gert-Jan
van Der Maarel, Silvere
Raz, Vered

January 2011

International audienceABSTRACT: Oculopharyngeal muscular dystrophy (OPMD) is a late-onset progressiv...

Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients

Anvar Seyed
't Hoen Peter AC
Venema Andrea
van der Sluijs Barbara
van Engelen Baziel
Snoeck Marc
Vissing John
Trollet Capucine
Dickson George
Chartier Aymeric
Simonelig Martine
van Ommen Gert-Jan B
van der Maarel Silvere M
Raz Vered

April 2011

Abstract Oculopharyngeal muscular dystrophy (OPMD) is a late-onset progressive muscle disorder cause...

RESEARCH ARTICLE Metabolic Dysfunction and Altered Mitochondrial Dynamics in the Utrophin- Dystrophin Deficient Mouse Model of Duchenne Muscular Dystrophy

Meghna Pant
Danesh H. Sopariwala
Naresh C. Bal
Jeovanna Lowe
A. Delfín
Jill Rafael-fortney
Muthu Periasamy

August 2016

The utrophin-dystrophin deficient (DKO) mouse model has been widely used to under-stand the progress...

Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres

Trollet, C.
Anvar, S.Y.
Venema, A.
Hargreaves, I.P.
Foster, Keith
Vignaud, A.
Ferry, A.
Negroni, E
Hourde, C.
Baraibar, M.A.
t'Hoen, P.A.C.
Davies, J.E.
Rubinsztein, D.C.
Heales, S.J.
Mouly, V.
van der Maarel, S.M.
Butler-Browne, G.
Raz, V.
Dickson, G.

January 2010

Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset disorder characterized by ptosis, dyspha...

Investigation of molecular mechanisms underlying Oculopharyngeal Muscular Dystrophy (OPMD)

Messaed, Christiane.

January 2007

Oculopharyngeal Muscular Dystrophy (OPMD) is a late-onset dominant/recessive myopathy caused by the ...

Gene expression analysis in myotonic dystrophy: indications for a common molecular pathogenic pathway in DM1 and DM2.

BOTTA, ANNALISA
AMATI, FRANCESCA
NOVELLI, GIUSEPPE
Vallo, L
Rinaldi, F
Bonifazi, E
BIANCOLELLA, MICHELA
Gambardella, S
Mancinelli, E
Angelini, C
Meola, G

January 2007

An RNA gain-of-function of expanded transcripts is the most accredited molecular mechanism for myoto...

Nuclear entrapment and extracellular depletion of PCOLCE is associated with muscle degeneration in oculopharyngeal muscular dystrophy.

Raz, Vered
Sterrenburg, Ellen
Routledge, Samantha
Venema, Andrea
van Der Sluijs, Barbara
Trollet, Capucine
Dickson, George
van Engelen, Baziel
van Der Maarel, Silvère
Antoniou, Michael

July 2013

International audienceBACKGROUND: Muscle fibrosis characterizes degenerated muscles in muscular dyst...

The Interplay of Mitophagy and Inflammation in Duchenne Muscular Dystrophy

Andrea L. Reid
Matthew S. Alexander

July 2021

Duchenne muscular dystrophy (DMD) is an X-linked neuromuscular disease caused by a pathogenic disrup...

A revised model for mitochondrial dysfunction in Duchenne muscular dystrophy

Ai Vu Hong
Mathilde Sanson
Isabelle Richard
David Israeli

September 2021

We recetly identified a signaling pathway that links the upregulation of miR-379 with a mitochondria...

Molecular model of OPMD.

Aymeric Chartier (713911)
Pierre Klein (713912)
Stéphanie Pierson (713913)
Nicolas Barbezier (713914)
Teresa Gidaro (688497)
François Casas (93372)
Steven Carberry (713915)
Paul Dowling (713916)
Laurie Maynadier (713917)
Maëlle Bellec (713918)
Martine Oloko (713919)
Claude Jardel (713920)
Bodo Moritz (146655)
George Dickson (123905)
Vincent Mouly (75137)
Kay Ohlendieck (1235)
Gillian Butler-Browne (75143)
Capucine Trollet (713921)
Martine Simonelig (713922)

March 2015

<p>The first molecular defect in OPMD would be a general decrease in the cleavage/polyadenylation re...

Mitochondrial Dysfunction Reveals the Role of mRNA Poly(A) Tail Regulation in Oculopharyngeal Muscular Dystrophy Pathogenesis

Chartier, Aymeric
Klein, Pierre
Pierson, Stéphanie
Barbezier, Nicolas
Gidaro, Teresa
Casas, François
Carberry, Steven
Dowling, Paul
Maynadier, Laurie
Bellec, Maëlle
Oloko, Martine
Jardel, Claude
Moritz, Bodo
Dickson, George
Mouly, Vincent
Ohlendieck, Kay
Butler-Browne, Gillian
Trollet, Capucine
Simonelig, Martine

March 2015

International audienceOculopharyngeal muscular dystrophy (OPMD), a late-onset disorder characterized...

Mitochondrial abnormalities in genetically assessed oculopharyngeal muscular dystrophy.

GAMBELLI S.
DE STEFANO R.
FRANCI M.
BRUTTINI F.
MALANDRINI, ALESSANDRO
GINANNESCHI, FEDERICA
BERTI, GIANNA
CARDAIOLI, ELENA
SALVADORI, CLAUDIO
MARI, FRANCESCA
ROSSI, ALESSANDRO
FEDERICO, ANTONIO
RENIERI, ALESSANDRA

January 2004

We report a family with a clinical diagnosis of oculopharyngeal muscular dystrophy in which muscle b...

Oculopharyngeal muscular dystrophy: Recent advances in the understanding of the molecular pathogenic mechanisms and treatment strategies

Abu-Baker, Aida
Rouleau, Guy A.

February 2007

AbstractOculopharyngeal muscular dystrophy (OPMD) is an adult-onset disorder characterized by progre...

RESEARCH ARTICLE Defects in Mitochondrial ATP Synthesis in Dystrophin-Deficient Mdx Skeletal Muscles May Be Caused by Complex I Insufficiency

Emma Rybalka
Cara A. Timpani
Matthew B. Cooke
Andrew D. Williams
Alan Hayes

August 2016

Duchenne Muscular Dystrophy is a chronic, progressive and ultimately fatal skeletal muscle wasting d...

Nuclear entrapment and extracellular depletion of PCOLCE is associated with muscle degeneration in oculopharyngeal muscular dystrophy

Raz, V.
Sterrenburg, E.
Routledge, S.
Venema, A.
Sluijs, B.M. van der
Trollet, C.
Dickson, G.
Engelen, B.G.M. van
Maarel, S.M. van der
Antoniou, M.N.

January 2013

Contains fulltext : 181501.pdf (publisher's version ) (Open Access)BACKGROUND: Mus...

Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients.

Anvar, Seyed, Yahya
T Hoen, Peter
Venema, Andrea
van Der Sluijs, Barbara
van Engelen, Baziel
Snoeck, Marc
Vissing, John
Trollet, Capucine
Dickson, George
Chartier, Aymeric
Simonelig, Martine
van Ommen, Gert-Jan
van Der Maarel, Silvere
Raz, Vered

January 2011

International audienceABSTRACT: Oculopharyngeal muscular dystrophy (OPMD) is a late-onset progressiv...

Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients

Anvar Seyed
't Hoen Peter AC
Venema Andrea
van der Sluijs Barbara
van Engelen Baziel
Snoeck Marc
Vissing John
Trollet Capucine
Dickson George
Chartier Aymeric
Simonelig Martine
van Ommen Gert-Jan B
van der Maarel Silvere M
Raz Vered

April 2011

Abstract Oculopharyngeal muscular dystrophy (OPMD) is a late-onset progressive muscle disorder cause...

RESEARCH ARTICLE Metabolic Dysfunction and Altered Mitochondrial Dynamics in the Utrophin- Dystrophin Deficient Mouse Model of Duchenne Muscular Dystrophy

Meghna Pant
Danesh H. Sopariwala
Naresh C. Bal
Jeovanna Lowe
A. Delfín
Jill Rafael-fortney
Muthu Periasamy

August 2016

The utrophin-dystrophin deficient (DKO) mouse model has been widely used to under-stand the progress...

Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres

Trollet, C.
Anvar, S.Y.
Venema, A.
Hargreaves, I.P.
Foster, Keith
Vignaud, A.
Ferry, A.
Negroni, E
Hourde, C.
Baraibar, M.A.
t'Hoen, P.A.C.
Davies, J.E.
Rubinsztein, D.C.
Heales, S.J.
Mouly, V.
van der Maarel, S.M.
Butler-Browne, G.
Raz, V.
Dickson, G.

January 2010

Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset disorder characterized by ptosis, dyspha...

Investigation of molecular mechanisms underlying Oculopharyngeal Muscular Dystrophy (OPMD)

Messaed, Christiane.

January 2007

Oculopharyngeal Muscular Dystrophy (OPMD) is a late-onset dominant/recessive myopathy caused by the ...

Gene expression analysis in myotonic dystrophy: indications for a common molecular pathogenic pathway in DM1 and DM2.

BOTTA, ANNALISA
AMATI, FRANCESCA
NOVELLI, GIUSEPPE
Vallo, L
Rinaldi, F
Bonifazi, E
BIANCOLELLA, MICHELA
Gambardella, S
Mancinelli, E
Angelini, C
Meola, G

January 2007

An RNA gain-of-function of expanded transcripts is the most accredited molecular mechanism for myoto...

Nuclear entrapment and extracellular depletion of PCOLCE is associated with muscle degeneration in oculopharyngeal muscular dystrophy.

Raz, Vered
Sterrenburg, Ellen
Routledge, Samantha
Venema, Andrea
van Der Sluijs, Barbara
Trollet, Capucine
Dickson, George
van Engelen, Baziel
van Der Maarel, Silvère
Antoniou, Michael

July 2013

International audienceBACKGROUND: Muscle fibrosis characterizes degenerated muscles in muscular dyst...

The Interplay of Mitophagy and Inflammation in Duchenne Muscular Dystrophy

Andrea L. Reid
Matthew S. Alexander

July 2021

Duchenne muscular dystrophy (DMD) is an X-linked neuromuscular disease caused by a pathogenic disrup...

A revised model for mitochondrial dysfunction in Duchenne muscular dystrophy

Ai Vu Hong
Mathilde Sanson
Isabelle Richard
David Israeli

September 2021

We recetly identified a signaling pathway that links the upregulation of miR-379 with a mitochondria...

Molecular model of OPMD.

Aymeric Chartier (713911)
Pierre Klein (713912)
Stéphanie Pierson (713913)
Nicolas Barbezier (713914)
Teresa Gidaro (688497)
François Casas (93372)
Steven Carberry (713915)
Paul Dowling (713916)
Laurie Maynadier (713917)
Maëlle Bellec (713918)
Martine Oloko (713919)
Claude Jardel (713920)
Bodo Moritz (146655)
George Dickson (123905)
Vincent Mouly (75137)
Kay Ohlendieck (1235)
Gillian Butler-Browne (75143)
Capucine Trollet (713921)
Martine Simonelig (713922)

March 2015

<p>The first molecular defect in OPMD would be a general decrease in the cleavage/polyadenylation re...

RESEARCH ARTICLE Mitochondrial Dysfunction Reveals the Role of mRNA Poly(A) Tail Regulation in Oculopharyngeal Muscular Dystrophy Pathogenesis

Abstract

Extracted data

RESEARCH ARTICLE Mitochondrial Dysfunction Reveals the Role of mRNA Poly(A) Tail Regulation in Oculopharyngeal Muscular Dystrophy Pathogenesis

Abstract

Extracted data

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