The Journal of Cell Biology
JCB
and Joshua R.Sanes and itsneuromuscular andmyotendinous junctions isoforms of Tyrosine-phosphorylated andnonphosphorylated the sarcolemma ofmusclecells.Micelacking sarcoglycans ( produces two main nous junctions(MTJs).Innormalmuscle,alternative splicing junctions (NMJs)and,asshown here,abnormalmyotendi- muscular dystrophy, defectsinmaturation ofneuromuscular dystrophin, utrophin,dystroglycans( include (Ervasti andCampbell,1993).ConstituentsoftheDGC intracellular cytoskeletonandtheextracellularbasallamina forms amultimolecular,transmembranelinkbetweenthe found throughoutthesarcolemmaofskeletalmusclecells, whichis The dystrophin–glycoproteincomplex(DGC),* common NH 3 1 dominant isoforminextrajunctional regions,andisalso phosphorylated, isconcentrated attheNMJsandMTJs. Introduction R. Mark Grady, finding thatmutationsingenesencodingnumerousDGC al., 2002).InterestintheDGCstemmedoriginallyfrom al., 1990;YoshidaandOzawa,forreviewseeBlake et rBTX, rhodamine junction; NMJ,neuromuscular nNOS,nitricoxidesynthase; dystrobrevin; DGC,dystrophin–glycoprotein complex;MTJ,myotendinous *Abbreviations usedinthispaper: AChR,acetylcholinereceptor;DB, [email protected] Louis, MO63110.Tel.:(314)286-2796.Fax:286-2892.E-mail: ington UniversitySchoolofMedicine,PediatricResearchBldg.,St. Address correspondencetoR.MarkGrady,Dept.ofPediatrics,Wash- http://www.jcb.org/cgi/doi/10.1083/jcb.200209045 The Journal ofCellBiology junction; neuromuscular junction junction; neuromuscular Key words: myotendinous dystrobrevin; dystrophin;musculardystrophy;
Department ofCellandDevelopmentalDepartment Biology, State University ofNew York Upstate Medical University, Syracuse, NY13210 ofPediatricsDepartment and St. Louis,MO63110
DB2, which isnottyrosinephosphorylated,thepre- DB1, whose COOH-terminalextensioncanbetyrosine 2), dystrobrevins(DBs; TheRockefeller University Press, 0021-9525
Article
dystrophin–glycoprotein complex,isfoundthroughout -Dystrobrevin (DB),acytoplasmic componentofthe
2
-terminal butdistinctCOOH-terminaldomains.