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Items: 16


Titin splicing regulates cardiotoxicity associated with calpain 3 gene therapy for limb-girdle muscular dystrophy type 2A.

Lostal W, Roudaut C, Faivre M, Charton K, Suel L, Bourg N, Best H, Smith JE, Gohlke J, Corre G, Li X, Elbeck Z, Knöll R, Deschamps JY, Granzier H, Richard I.

Sci Transl Med. 2019 Nov 27;11(520). pii: eaat6072. doi: 10.1126/scitranslmed.aat6072.


Exploiting the CRISPR/Cas9 system to study alternative splicing in vivo: application to titin.

Charton K, Suel L, Henriques SF, Moussu JP, Bovolenta M, Taillepierre M, Becker C, Lipson K, Richard I.

Hum Mol Genet. 2016 Oct 15;25(20):4518-4532. doi: 10.1093/hmg/ddw280.


CAPN3-mediated processing of C-terminal titin replaced by pathological cleavage in titinopathy.

Charton K, Sarparanta J, Vihola A, Milic A, Jonson PH, Suel L, Luque H, Boumela I, Richard I, Udd B.

Hum Mol Genet. 2015 Jul 1;24(13):3718-31. doi: 10.1093/hmg/ddv116. Epub 2015 Apr 15.


Cis-splicing and translation of the pre-trans-splicing molecule combine with efficiency in spliceosome-mediated RNA trans-splicing.

Monjaret F, Bourg N, Suel L, Roudaut C, Le Roy F, Richard I, Charton K.

Mol Ther. 2014 Jun;22(6):1176-1187. doi: 10.1038/mt.2014.35. Epub 2014 Mar 12.


Restriction of calpain3 expression to the skeletal muscle prevents cardiac toxicity and corrects pathology in a murine model of limb-girdle muscular dystrophy.

Roudaut C, Le Roy F, Suel L, Poupiot J, Charton K, Bartoli M, Richard I.

Circulation. 2013 Sep 3;128(10):1094-104. doi: 10.1161/CIRCULATIONAHA.113.001340. Epub 2013 Aug 1.


Lack of correlation between outcomes of membrane repair assay and correction of dystrophic changes in experimental therapeutic strategy in dysferlinopathy.

Lostal W, Bartoli M, Roudaut C, Bourg N, Krahn M, Pryadkina M, Borel P, Suel L, Roche JA, Stockholm D, Bloch RJ, Levy N, Bashir R, Richard I.

PLoS One. 2012;7(5):e38036. doi: 10.1371/journal.pone.0038036. Epub 2012 May 29.


A new pathway encompassing calpain 3 and its newly identified substrate cardiac ankyrin repeat protein is involved in the regulation of the nuclear factor-κB pathway in skeletal muscle.

Laure L, Danièle N, Suel L, Marchand S, Aubert S, Bourg N, Roudaut C, Duguez S, Bartoli M, Richard I.

FEBS J. 2010 Oct;277(20):4322-37. doi: 10.1111/j.1742-4658.2010.07820.x. Epub 2010 Sep 22.


Cardiac ankyrin repeat protein is a marker of skeletal muscle pathological remodelling.

Laure L, Suel L, Roudaut C, Bourg N, Ouali A, Bartoli M, Richard I, Danièle N.

FEBS J. 2009 Feb;276(3):669-84. doi: 10.1111/j.1742-4658.2008.06814.x.


NF-kappaB-dependent expression of the antiapoptotic factor c-FLIP is regulated by calpain 3, the protein involved in limb-girdle muscular dystrophy type 2A.

Benayoun B, Baghdiguian S, Lajmanovich A, Bartoli M, Daniele N, Gicquel E, Bourg N, Raynaud F, Pasquier MA, Suel L, Lochmuller H, Lefranc G, Richard I.

FASEB J. 2008 May;22(5):1521-9. Epub 2007 Dec 11.


Safety and efficacy of AAV-mediated calpain 3 gene transfer in a mouse model of limb-girdle muscular dystrophy type 2A.

Bartoli M, Roudaut C, Martin S, Fougerousse F, Suel L, Poupiot J, Gicquel E, Noulet F, Danos O, Richard I.

Mol Ther. 2006 Feb;13(2):250-9. Epub 2005 Nov 14.


Six and Eya expression during human somitogenesis and MyoD gene family activation.

Fougerousse F, Durand M, Lopez S, Suel L, Demignon J, Thornton C, Ozaki H, Kawakami K, Barbet P, Beckmann JS, Maire P.

J Muscle Res Cell Motil. 2002;23(3):255-64.


Sequential expression of genes involved in muscular dystrophies during human development.

Durand M, Suel L, Barbet JP, Beckmann JS, Fougerousse F.

Morphologie. 2002 Jun;86(273):9-12.


Loss of calpain 3 proteolytic activity leads to muscular dystrophy and to apoptosis-associated IkappaBalpha/nuclear factor kappaB pathway perturbation in mice.

Richard I, Roudaut C, Marchand S, Baghdiguian S, Herasse M, Stockholm D, Ono Y, Suel L, Bourg N, Sorimachi H, Lefranc G, Fardeau M, Sébille A, Beckmann JS.

J Cell Biol. 2000 Dec 25;151(7):1583-90.


Calpain3 expression during human cardiogenesis.

Fougerousse F, Anderson LV, Delezoide AL, Suel L, Durand M, Beckmann JS.

Neuromuscul Disord. 2000 Jun;10(4-5):251-6.


Human-mouse differences in the embryonic expression patterns of developmental control genes and disease genes.

Fougerousse F, Bullen P, Herasse M, Lindsay S, Richard I, Wilson D, Suel L, Durand M, Robson S, Abitbol M, Beckmann JS, Strachan T.

Hum Mol Genet. 2000 Jan 22;9(2):165-73. Erratum in: Hum Mol Genet 2000 Mar 1;9(4):659.


Expression of genes (CAPN3, SGCA, SGCB, and TTN) involved in progressive muscular dystrophies during early human development.

Fougerousse F, Durand M, Suel L, Pourquié O, Delezoide AL, Romero NB, Abitbol M, Beckmann JS.

Genomics. 1998 Mar 1;48(2):145-56.


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