DUX4MonoclonalAntibody
CatalogNo.:57101(cloneP2B1)Size:100uginPBS,pH7.4.PurifiedbyProteinGaffinitychromatography.
Facioscapulohumeralmusculardystrophy(FSHD)iscausedbythedeletionofasubsetofD4Z4macro-satelliterepeatsonchromosome4.Eachrepeatcontainsaretrogeneencodingthedouble-homeoboxfactorDUX4.DUX4expressionisepi-geneticallysuppressedindifferentiatedtissues,andtheresidualDUX4transcriptsaresplicedtoremovethecarboxyterminaldomainthathasbeenassociatedwithcelltoxicity.InFSHDindividuals,theexpressionofthefull-lengthDUX4transcriptisnotcompletelysuppressedinskeletalmuscleandpossIBLyotherdifferentiatedtissues.
Antigen:C-terminal76aminoacidsofDUX4withglutathione-s-transferase(gst)tag.
HostSpecies:Mouse
AntibodyClass:IgG1
ThisantibodyrecognizeshumanDUX4.Itdoesnotcross-reactwithDUX4c.
SniderLetal2010PLoSGenetics6:1-14.
APPLICATIONSImmunohistochemistry
FrozensectionsofhumantestisstainedwithP2B1.
Immunofluorescence
C2C12myoblaststransfectedwithpCS2+DUX4stainedwithP2B1.CounterstainedwithDAPIfornuclei.
Seereferencebelowforproceduraldetails.Endusershoulddetermineoptimalconcentrationsfortheirapplications.
DILUTIONINSTRUCTIONSDiluteinPBSormediumwhichisidenticaltothatusedintheassaysystem.
STORAGEANDSTABILITYThisantibodyisstableforatleastone(1)yearat-20oC.
Forinvitroinvestigationaluseonly.Notintendedfordiagnosticortherapeuticapplications.
