DUX4MonoclonalAntibody

ORDERINGINFORMATION

CatalogNo.:57101(cloneP2B1)Size:100uginPBS,pH7.4.PurifiedbyProteinGaffinitychromatography.

BACKGROUND

Facioscapulohumeralmusculardystrophy(FSHD)iscausedbythedeletionofasubsetofD4Z4macro-satelliterepeatsonchromosome4.Eachrepeatcontainsaretrogeneencodingthedouble-homeoboxfactorDUX4.DUX4expressionisepi-geneticallysuppressedindifferentiatedtissues,andtheresidualDUX4transcriptsaresplicedtoremovethecarboxyterminaldomainthathasbeenassociatedwithcelltoxicity.InFSHDindividuals,theexpressionofthefull-lengthDUX4transcriptisnotcompletelysuppressedinskeletalmuscleandpossIBLyotherdifferentiatedtissues.

SPECIFICATIONSUMMARY

Antigen:C-terminal76aminoacidsofDUX4withglutathione-s-transferase(gst)tag.

HostSpecies:Mouse

AntibodyClass:IgG1

SPECIFICITY

ThisantibodyrecognizeshumanDUX4.Itdoesnotcross-reactwithDUX4c.

REFERENCE

SniderLetal2010PLoSGenetics6:1-14.

APPLICATIONS

Immunohistochemistry

FrozensectionsofhumantestisstainedwithP2B1.

Immunofluorescence

C2C12myoblaststransfectedwithpCS2+DUX4stainedwithP2B1.CounterstainedwithDAPIfornuclei.

Seereferencebelowforproceduraldetails.Endusershoulddetermineoptimalconcentrationsfortheirapplications.

DILUTIONINSTRUCTIONS

DiluteinPBSormediumwhichisidenticaltothatusedintheassaysystem.

STORAGEANDSTABILITY

Thisantibodyisstableforatleastone(1)yearat-20oC.

Forinvitroinvestigationaluseonly.Notintendedfordiagnosticortherapeuticapplications.

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