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

Capucine Trollet; Seyed Yahya Anvar; Andrea Venema; Iain P. Hargreaves; Keith Foster; Alban Vignaud; Arnaud Ferry; Elisa Negroni; Christophe Hourde; Martin A. Baraibar; Peter A. C. 't Hoen; Janet E. Davies; David C. Rubinsztein; Simon J. Heales; Vincent Mouly; Silvere M. van der Maarel; Gillian Butler-Browne; Vered Raz; George Dickson

doi:10.1093/hmg/ddq098

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

Capucine Trollet
, Seyed Yahya Anvar
, Andrea Venema
, Iain P. Hargreaves
, Keith Foster
, Alban Vignaud
, Arnaud Ferry
, Elisa Negroni
, Christophe Hourde
, Martin A. Baraibar
, Peter A. C. 't Hoen
, Janet E. Davies
, David C. Rubinsztein
, Simon J. Heales
, Vincent Mouly
, Silvere M. van der Maarel
, Gillian Butler-Browne
, Vered Raz
, George Dickson

Research output: Contribution to journal › Article › peer-review

Original language	English
Pages (from-to)	2191-2207
Number of pages	17
Journal	Human Molecular Genetics
Volume	19
Issue number	11
Early online date	5 Mar 2010
DOIs	https://doi.org/10.1093/hmg/ddq098
Publication status	Published - 1 Jun 2010

Keywords

SKELETAL-MUSCLE ATROPHY
NUCLEAR INCLUSIONS
GENE-EXPRESSION
TRANSGENIC MICE
UBIQUITIN-PROTEASOME
AGGREGATE FORMATION
PROTEIN PABPN1
IN-VIVO
INACTIVATION
EXPANSIONS

Access to Document

10.1093/hmg/ddq098

http://hmg.oxfordjournals.org/content/19/11/2191

Cite this

Trollet, C., Anvar, S. Y., Venema, A., Hargreaves, I. P., Foster, K., 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. (2010). Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres. Human Molecular Genetics, 19(11), 2191-2207. https://doi.org/10.1093/hmg/ddq098

@article{bb8afacb304e4e9aa888c98461b19d46,

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

keywords = "SKELETAL-MUSCLE ATROPHY, NUCLEAR INCLUSIONS, GENE-EXPRESSION, TRANSGENIC MICE, UBIQUITIN-PROTEASOME, AGGREGATE FORMATION, PROTEIN PABPN1, IN-VIVO, INACTIVATION, EXPANSIONS",

author = "Capucine Trollet and Anvar, \{Seyed Yahya\} and Andrea Venema and Hargreaves, \{Iain P.\} and Keith Foster and Alban Vignaud and Arnaud Ferry and Elisa Negroni and Christophe Hourde and Baraibar, \{Martin A.\} and \{'t Hoen\}, \{Peter A. C.\} and Davies, \{Janet E.\} and Rubinsztein, \{David C.\} and Heales, \{Simon J.\} and Vincent Mouly and \{van der Maarel\}, \{Silvere M.\} and Gillian Butler-Browne and Vered Raz and George Dickson",

year = "2010",

month = jun,

day = "1",

doi = "10.1093/hmg/ddq098",

language = "English",

volume = "19",

pages = "2191--2207",

journal = "Human Molecular Genetics",

issn = "0964-6906",

publisher = "Oxford University Press",

number = "11",

}

Trollet, C, Anvar, SY, Venema, A, Hargreaves, IP, Foster, K, Vignaud, A, Ferry, A, Negroni, E, Hourde, C, Baraibar, MA, 't Hoen, PAC, Davies, JE, Rubinsztein, DC, Heales, SJ, Mouly, V, van der Maarel, SM, Butler-Browne, G, Raz, V & Dickson, G 2010, 'Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres', Human Molecular Genetics, vol. 19, no. 11, pp. 2191-2207. https://doi.org/10.1093/hmg/ddq098

Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres. / Trollet, Capucine; Anvar, Seyed Yahya; Venema, Andrea et al.
In: Human Molecular Genetics, Vol. 19, No. 11, 01.06.2010, p. 2191-2207.

Research output: Contribution to journal › Article › peer-review

TY - JOUR

T1 - Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres

AU - Trollet, Capucine

AU - Anvar, Seyed Yahya

AU - Venema, Andrea

AU - Hargreaves, Iain P.

AU - Foster, Keith

AU - Vignaud, Alban

AU - Ferry, Arnaud

AU - Negroni, Elisa

AU - Hourde, Christophe

AU - Baraibar, Martin A.

AU - 't Hoen, Peter A. C.

AU - Davies, Janet E.

AU - Rubinsztein, David C.

AU - Heales, Simon J.

AU - Mouly, Vincent

AU - van der Maarel, Silvere M.

AU - Butler-Browne, Gillian

AU - Raz, Vered

AU - Dickson, George

PY - 2010/6/1

Y1 - 2010/6/1

KW - SKELETAL-MUSCLE ATROPHY

KW - NUCLEAR INCLUSIONS

KW - GENE-EXPRESSION

KW - TRANSGENIC MICE

KW - UBIQUITIN-PROTEASOME

KW - AGGREGATE FORMATION

KW - PROTEIN PABPN1

KW - IN-VIVO

KW - INACTIVATION

KW - EXPANSIONS

U2 - 10.1093/hmg/ddq098

DO - 10.1093/hmg/ddq098

M3 - Article

SN - 0964-6906

VL - 19

SP - 2191

EP - 2207

JO - Human Molecular Genetics

JF - Human Molecular Genetics

IS - 11

ER -