Literature DB >> 27501221

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells.

Rui Liang1, Wei Dong2, Xiaopeng Shen1, Xiaoping Peng2, Angie G Aceves3, Yu Liu4.   

Abstract

Myotonic dystrophy 1 (DM1) is a common form of muscular dystrophy. Although several animal models have been established for DM1, myoblast cell models are still important because they offer an efficient cellular alternative for studying cellular and molecular events. Though C2C12 myoblast cells have been widely used to study myogenesis, resistance to gene transfection, or viral transduction, hinders research in C2C12 cells. Here, we describe an optimized protocol that includes daily maintenance, transfection and transduction procedures to introduce genes into C2C12 myoblasts and the induction of myocyte differentiation. Collectively, these procedures enable best transfection/transduction efficiencies, as well as consistent differentiation outcomes. The protocol described in establishing DM1 myoblast cell models would benefit the study of myotonic dystrophy, as well as other muscular diseases.

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Year:  2016        PMID: 27501221      PMCID: PMC5091708          DOI: 10.3791/54078

Source DB:  PubMed          Journal:  J Vis Exp        ISSN: 1940-087X            Impact factor:   1.355


  15 in total

1.  (CTG)n repeats markedly inhibit differentiation of the C2C12 myoblast cell line: implications for congenital myotonic dystrophy.

Authors:  S Bhagavati; S Bhagwati; S A Shafiq; W Xu
Journal:  Biochim Biophys Acta       Date:  1999-02-24

2.  The Mef2 transcription network is disrupted in myotonic dystrophy heart tissue, dramatically altering miRNA and mRNA expression.

Authors:  Auinash Kalsotra; Ravi K Singh; Priyatansh Gurha; Amanda J Ward; Chad J Creighton; Thomas A Cooper
Journal:  Cell Rep       Date:  2014-01-09       Impact factor: 9.423

3.  Serial passaging and differentiation of myogenic cells isolated from dystrophic mouse muscle.

Authors:  D Yaffe; O Saxel
Journal:  Nature       Date:  1977 Dec 22-29       Impact factor: 49.962

4.  The myotonic dystrophy expanded CUG repeat tract is necessary but not sufficient to disrupt C2C12 myoblast differentiation.

Authors:  J D Amack; M S Mahadevan
Journal:  Hum Mol Genet       Date:  2001-09-01       Impact factor: 6.150

5.  Celf1 regulates cell cycle and is partially responsible for defective myoblast differentiation in myotonic dystrophy RNA toxicity.

Authors:  Xiaoping Peng; Xiaopeng Shen; Xuanying Chen; Rui Liang; Alon R Azares; Yu Liu
Journal:  Biochim Biophys Acta       Date:  2015-04-15

6.  Cis and trans effects of the myotonic dystrophy (DM) mutation in a cell culture model.

Authors:  J D Amack; A P Paguio; M S Mahadevan
Journal:  Hum Mol Genet       Date:  1999-10       Impact factor: 6.150

Review 7.  Developmental insights into the pathology of and therapeutic strategies for DM1: Back to the basics.

Authors:  Anthony Chau; Auinash Kalsotra
Journal:  Dev Dyn       Date:  2015-01-13       Impact factor: 3.780

8.  Muscleblind proteins regulate alternative splicing.

Authors:  Thai H Ho; Nicolas Charlet-B; Michael G Poulos; Gopal Singh; Maurice S Swanson; Thomas A Cooper
Journal:  EMBO J       Date:  2004-07-15       Impact factor: 11.598

9.  Heart-specific overexpression of CUGBP1 reproduces functional and molecular abnormalities of myotonic dystrophy type 1.

Authors:  Misha Koshelev; Satyam Sarma; Roger E Price; Xander H T Wehrens; Thomas A Cooper
Journal:  Hum Mol Genet       Date:  2010-01-05       Impact factor: 6.150

10.  Molecular basis of myotonic dystrophy: expansion of a trinucleotide (CTG) repeat at the 3' end of a transcript encoding a protein kinase family member.

Authors:  J D Brook; M E McCurrach; H G Harley; A J Buckler; D Church; H Aburatani; K Hunter; V P Stanton; J P Thirion; T Hudson
Journal:  Cell       Date:  1992-02-21       Impact factor: 41.582

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  1 in total

1.  An improved method with high sensitivity and low background in detecting low β-galactosidase expression in mouse embryos.

Authors:  Xiaopeng Shen; Wenjing Bao; Wei Yu; Rui Liang; Bao Nguyen; Yu Liu
Journal:  PLoS One       Date:  2017-05-05       Impact factor: 3.240

  1 in total

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