Name: modeling myotonic dystrophy 1 in c2c12 myoblast cells
Uploaded: 2016-07-29T14:00:00
Description: Watch this Scientific Journal Video about Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells at JoVE.com

We thank Drs. Tom Cooper from the Baylor College of Medicine, Mani S. Mahadevan from the University of Wisconsin-Madison, and Didier Trono from the University of Geneva for reagents. This work is supported by a University of Houston startup fund (YL), American Heart Association grant (YL, 11SDG5260033), and the National Natural Science Foundation of China (XP, 81460047).

1. C2C12 Cell Culture <ol><li> Opprettholde C2C12 mus myoblaster i en 100 mm skål i vekstmedium (Dulbeccos modifiserte Eagles medium (DMEM)) supplert med 20% føtalt bovint serum, 100 U / ml penicillin, 100 ug / ml streptomycin, og 2 mM L-glutamin. Tillat C2C12 passert cellene til å bli ca. 50 - 60% sammenflytende. </li><li> Kast vekstmediet og vaskes C2C12-celler sammen med 3 ml romtemperatur fosfat-bufret saltvann (PBS). Fjern PBS og tilsett 500 mL 0,25% Trypsin-EDTA å løsne cellene. Sett platen i en 37 ° C, <s...

<ol>
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M., Chiu, C. P., Webster, C. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Cytoplasmic+activation+of+human+nuclear+genes+in+stable+heterocaryons.">Cytoplasmic activation of human nuclear genes in stable heterocaryons.</a> Cell. 32 (4), 1171-1180 (1983).</li><li>Peng, X., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Celf1+regulates+cell+cycle+and+is+partially+responsible+for+defective+myoblast+differentiation+in+myotonic+dystrophy+RNA+toxicity.">Celf1 regulates cell cycle and is partially responsible for defective myoblast differentiation in myotonic dystrophy RNA toxicity.</a> Biochim Biophys Acta. 1852 (7), 1490-1497 (2015).</li><li>Amack, J. D., Mahadevan, M. S. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=The+myotonic+dystrophy+expanded+CUG+repeat+tract+is+necessary+but+not+sufficient+to+disrupt+C2C12+myoblast+differentiation.">The myotonic dystrophy expanded CUG repeat tract is necessary but not sufficient to disrupt C2C12 myoblast differentiation.</a> Hum Mol Genet. 10 (18), 1879-1887 (2001).</li><li>Amack, J. D., Paguio, A. P., Mahadevan, M. S. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Cis+and+trans+effects+of+the+myotonic+dystrophy+(DM)+mutation+in+a+cell+culture+model.">Cis and trans effects of the myotonic dystrophy (DM) mutation in a cell culture model.</a> Hum Mol Genet. 8 (11), 1975-1984 (1999).</li><li>Bhagavati, S., Shafiq, S. A., Xu, W. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=(CTG)n+repeats+markedly+inhibit+differentiation+of+the+C2C12+myoblast+cell+line:+implications+for+congenital+myotonic+dystrophy.">(CTG)n repeats markedly inhibit differentiation of the C2C12 myoblast cell line: implications for congenital myotonic dystrophy.</a> Biochim Biophys Acta. 1453 (2), 221-229 (1999).</li><li>Amack, J. D., Mahadevan, M. S. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Myogenic+defects+in+myotonic+dystrophy.">Myogenic defects in myotonic dystrophy.</a> Dev Biol. 265 (2), 294-301 (2004).</li><li>Emerson, C. P., Sweeney, H. L. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=.">.</a> Methods in muscle biology. 52, (1997).</li><li>Ward, A. J., Rimer, M., Killian, J. M., Dowling, J. J., Cooper, T. A. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=CUGBP1+overexpression+in+mouse+skeletal+muscle+reproduces+features+of+myotonic+dystrophy+type+1.">CUGBP1 overexpression in mouse skeletal muscle reproduces features of myotonic dystrophy type 1.</a> Hum Mol Genet. 19 (18), 3614-3622 (2010).</li><li>Koshelev, M., Sarma, S., Price, R. E., Wehrens, X. H. T., Cooper, T. 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C2C12 cellelinje har blitt brukt som modell for å studere myogenesen 11-14. Disse cellene beholde en fibroblast-lignende utseende, sprer seg raskt i media inneholdende 20% føtalt bovint serum og lett skille i media inneholdende 2% hesteserum 15. Den raske vekst og differensiering er fordelaktige egenskaper i en myogenesen celle modell. Her viser vi bruken av plasmidet, retroviral og lentiviral vektorer for å innføre cDNA, 3&#39;-UTR, og shRNA inn C2C12 celler. De kritiske punktene for transfeks...

Myotonic dystrofi (DM) er en autosomal dominant sykdom som påvirker flere systemer, særlig hjerte- og skjelettmuskulatur 1. Det er to undertyper av denne sykdommen, DM1 og DM2. DM1 er mer vanlig, og har en mer alvorlig manifestasjon enn DM2 2. Den genetiske mutasjon underliggende DM1 er en utvidelse av CUG triplet gjentar som befinner seg i det 3&#39;-ikke-translaterte region (UTR) av DM-protein kinase-genet (DMPK) 3. Den CUG gjenta tall i upåvirket individer varierer fra 5 til 37. I motsetning til dette øker til mer enn 50, og noen ganger opp til flere tusen i DM1 pasienter 4. Som et resultat av RNA-bindende proteiner, slik som muscleblind liknende 1 (MBNL1), CUGBP, og Elav lignende familie 1 (Celf1), er misregulated. På grunn av den håndtering på de ekspanderte CUG gjentas, mister MBNL1 dens evne til å regulere alternativ spleising 5. Celf1, på den annen side blir oppregulert 6,7. Overekspresjon av Celf1 er assosiert med muskel tapog svakhet, som ikke er knyttet til tap av MBNL1 funksjon. Dyremodeller simulerer DM1-relaterte endringer, inkludert DMPK 3&#39;-UTR CUG utvidelse, tap av MBNL1, og overekspresjon av Celf1, har blitt etablert. Men modellering DM1 i myoblasts tilbyr et effektivt alternativ, spesielt for dissekere DM1-relaterte cellulære og molekylære hendelser. C2C12 myoblast cellelinjen ble først isolert fra skadet C3H mus muskel og mye brukt til å studere myogenic differensiering 8,9. C2C12 celler raskt sprer i føtalt bovint serum (FBS) -inneholdende media og lett gjennomgå differensiering når FBS er oppbrukt. Likevel, ved hjelp av denne myoblast differensiering modellen presenterer to utfordringer: C2C12 cellene er ofte resistente mot genet transfeksjon / viral transduksjon; og små variasjoner i celledifferensiering håndtering og fremgangsmåte kan føre til markerte endringer i myotube formasjonen. Vår lab bruker rutinemessig C2C12 myoblasts som acell modell og har utviklet protokoller som effektivt leverer gener ved plasmid transfeksjon, retroviral transduksjon, og lentiviral transduksjon inn C2C12 cellelinje 10. I videoen viser vi de optimaliserte prosedyrer for trans / transducing C2C12 celler og opprettholde differensiering konsistens i å etablere DM1 myoblast modeller.

<table border="0" cellpadding="0" cellspacing="0" width="804">
	<tbody>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">DMEM, high glucose</td>
			<td style="width:112px;">Life Technologies</td>
			<td style="width:151px;">11965-084</td>
			<td style="width:325px;">for culture medium</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">Fetal Bovine Serum - Premium</td>
			<td style="width:112px;">Atlanta Biologicals</td>
			<td style="width:151px;">S11150</td>
			<td>for culture medium</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">Penicillin-Streptomycin-Glutamine (100X)</td>
			<td style="width:112px;">Life Technologies</td>
			<td style="width:151px;">10378-016</td>
			<td>for culture medium</td>
		</tr>
		<tr height="21">
			<td height="21" style="height:21px;width:216px;">Insulin from bovine pancreas</td>
			<td style="width:112px;">Sigma Aldrich</td>
			<td style="width:151px;">I6634-100MG</td>
			<td>for differentiation medium</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">equine serum</td>
			<td style="width:112px;">Atlanta Biologicals</td>
			<td style="width:151px;">S12150</td>
			<td>for differentiation medium</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">FuGENE&#160;HD&#160;Transfection Reagent</td>
			<td style="width:112px;">Promega</td>
			<td style="width:151px;">E2311&#160;</td>
			<td>for transfection</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">G418 sulfate&#160;</td>
			<td style="width:112px;">Gold Biotechnology&#160;</td>
			<td style="width:151px;">G-418-10</td>
			<td>for drug resistant selection</td>
		</tr>
		<tr height="21">
			<td height="21" style="height:21px;width:216px;">Puromycin dihydrochloride</td>
			<td style="width:112px;">Sigma Aldrich</td>
			<td style="width:151px;">sc-108071</td>
			<td>for drug resistant selection</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">NuPAGE Novex 4-12% Bis-Tris Protein Gels, 1.0 mm, 15 well</td>
			<td style="width:112px;">Life Technologies</td>
			<td style="width:151px;">NP0323BOX</td>
			<td>for western blot</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">NuPAGE Transfer Buffer (20X)</td>
			<td style="width:112px;">Life Technologies</td>
			<td style="width:151px;">NP00061</td>
			<td>for western blot</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">NuPAGE MES SDS Running Buffer (20X)</td>
			<td style="width:112px;">Life Technologies</td>
			<td style="width:151px;">NP0002</td>
			<td>for western blot</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">Amersham Protran Supported 0.2 NC, 300mmx4m</td>
			<td style="width:112px;">GE healthcare life science</td>
			<td style="width:151px;">10600015</td>
			<td>for western blot</td>
		</tr>
		<tr height="63">
			<td height="63" style="height:63px;width:216px;">MF 20</td>
			<td style="width:112px;">Developmental Hybridoma Bank</td>
			<td style="width:151px;">MF 20</td>
			<td>primary Ab for immunostaining</td>
		</tr>
		<tr height="63">
			<td height="63" style="height:63px;width:216px;">Goat anti-Mouse IgG (H+L) Secondary Antibody, Texas Red-X conjugate</td>
			<td style="width:112px;">Thermo Fisher Scientific</td>
			<td style="width:151px;">T-862</td>
			<td>secondary Ab for immunostaining</td>
		</tr>
		<tr height="21">
			<td height="21" style="height:21px;width:216px;">One step qRT-PCR MasterMix</td>
			<td style="width:112px;">AnaSpec</td>
			<td style="width:151px;">05-QPRT-032X</td>
			<td>for qRT-PCR</td>
		</tr>
		<tr height="21">
			<td height="21" style="height:21px;width:216px;">TriPure Isolation Reagent</td>
			<td style="width:112px;">Roche</td>
			<td style="width:151px;">11667165001</td>
			<td>for RNA isolation</td>
		</tr>
		<tr height="21">
			<td height="21" style="height:21px;width:216px;">CUG-BP1 Antibody (3B1)</td>
			<td style="width:112px;">santa cruz</td>
			<td style="width:151px;">sc-20003</td>
			<td>primary Ab western blot</td>
		</tr>
		<tr height="21">
			<td height="21" style="height:21px;width:216px;">Actin Antibody</td>
			<td style="width:112px;">santa cruz</td>
			<td style="width:151px;">sc-1615</td>
			<td>goat polyclonal IgG for loading control</td>
		</tr>
		<tr height="21">
			<td height="21" style="height:21px;width:216px;">293T Ecopack</td>
			<td style="width:112px;">Clontech</td>
			<td style="width:151px;">631507</td>
			<td>cells for retrovirus preparation</td>
		</tr>
		<tr height="21">
			<td height="21" style="height:21px;width:216px;">pMSCV-puro</td>
			<td style="width:112px;">Clontech</td>
			<td style="width:151px;">634401</td>
			<td>empty retroviral vector for retrovirus preparation</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">pMSCV-Celf1Flag-puro</td>
			<td style="width:112px;">house-constructed</td>
			<td style="width:151px;">not available</td>
			<td>retroviral vector encoding Celf1Flag, used in retrovirus preparation</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">psPAX2</td>
			<td style="width:112px;">gift from Didier Trono</td>
			<td style="width:151px;">not available</td>
			<td>for lentivirus preparation</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">pMD2.G</td>
			<td style="width:112px;">gift from Didier Trono</td>
			<td style="width:151px;">not available</td>
			<td>for lentivirus preparation</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">GFP-CUG5</td>
			<td style="width:112px;">gift from M.S. Mahadevan</td>
			<td style="width:151px;">not available</td>
			<td>details in reference 10&#160;</td>
		</tr>
		<tr height="42">
			<td height="42" style="height:42px;width:216px;">GFP- CUG200</td>
			<td style="width:112px;">gift from M.S. Mahadevan</td>
			<td style="width:151px;">not available</td>
			<td>details in reference 10&#160;</td>
		</tr>
		<tr height="21">
			<td height="21" style="height:21px;width:216px;">Triton&#160;X-100</td>
			<td style="width:112px;">Sigma Aldrich</td>
			<td style="width:151px;">X100</td>
			<td>for immunostaining</td>
		</tr>
		<tr height="21">
			<td height="21" style="height:21px;width:216px;">paraformaldehyde</td>
			<td style="width:112px;">Sigma Aldrich</td>
			<td style="width:151px;">P6148</td>
			<td>for immunostaining</td>
		</tr>
		<tr height="21">
			<td height="21" style="height:21px;width:216px;">TWEEN&#160;20</td>
			<td style="width:112px;">Sigma Aldrich</td>
			<td style="width:151px;">P9416</td>
			<td>for immunostaining</td>
		</tr>
		<tr height="21">
			<td height="21" style="height:21px;width:216px;">DAPI</td>
			<td style="width:112px;">Sigma Aldrich</td>
			<td style="width:151px;">D9542</td>
			<td>for immunostaining</td>
		</tr>
	</tbody>
</table>

modeling myotonic dystrophy 1 in c2c12 myoblast cells

Myotonic dystrofi 1 (DM1) er en vanlig form for muskeldystrofi. Selv om det er etablert flere dyremodeller for DM1, myoblast cellemodeller er fortsatt viktig, fordi de tilbyr en effektiv cellulær alternativ for å studere cellulære og molekylære hendelser. Selv om C2C12 myoblast celler har blitt mye brukt til å studere myogenese, motstand mot gen transfeksjon eller viral transduksjon, hindrer forskning i C2C12-celler. Her beskriver vi en optimalisert protokoll som inkluderer daglig vedlikehold, transfeksjon og transduksjon prosedyrer for å innføre gener i C2C12 myoblasts og induksjon av myocyte differensiering. Sammen er disse fremgangsmåtene gjør det mulig beste transfeksjon / overføringseffektivitet, så vel som differensierings konsistente resultater. Den protokoll beskrevet i etablering av DM1 myoblast cellemodeller som vil dra studiet av myotonisk dystrofi, så vel som andre muskelsykdommer.

C2C12 celler ble transfektert med GFP-CUG5 eller GFP-CUG200. Etter medikamentresistens seleksjon, ble stabile bassenger etablert, som kan visualiseres ved GFP uttrykket (figur 1A). Myotube dannelse i de differensierte myoblaster ble påvist ved myosin tungkjede farging 10 (figur 1B). Kvantifisering av myotube formasjonen viste at fusjons indeksene ble redusert fra 35,4 ± 4,1% til 2,6 ± 1,1% og myotube områder ble redusert fra 35,6 ± 2,2% t...

Watch this Scientific Journal Video about Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells at JoVE.com

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells

I denne protokollen, presenterer vi de prosedyrer i å etablere myotonic dystrofi 1 myoblast modeller, inkludert optimalisert C2C12 celle vedlikehold, genet transfeksjon / transduksjon, og myocyte differensiering.

Modellering Myotonic dystrofi 1 i C2C12 Myoblast Cells

Myotonic dystrophy 1 (DM1) is a common form of muscular dystrophy. Although several animal models have been established for DM1, myoblast cell models are ...

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Multi-exon Skipping Using Cocktail Antisense Oligonucleotides in the Canine X-linked Muscular Dystrophy

Multi-exon Hoppe Bruke Cocktail Antisenseoligonukleotider i Canine X-linked muskeldystrofi

Duchenne muscular dystrophy (DMD) is one of the most common lethal genetic diseases worldwide, caused by mutations in the dystrophin (DMD) gene. Exon ...

Depletion of Mouse Cells from Human Tumor Xenografts Significantly Improves Downstream Analysis of Target Cells

Nedbryting av museceller fra menneske tumorxenotransplantater forbedrer Nedstrøms Analyse av Target Cells

The use of in vitro cell line models for cancer research has been a useful tool. However, it has been shown that these models fail to reliably mimic ...

Evaluation of Exon Inclusion Induced by Splice Switching Antisense Oligonucleotides in SMA Patient Fibroblasts

Evaluering av ekson inkludering av skjøte bytte Antisense Oligonucleotides i SMA pasienten fibroblaster

Spinal muscular atrophy (SMA), a lethal neurological disease caused by the loss of SMN1, presents a unique case in the field of antisense oligonucleotide ...