Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrix.
The primary sequence of two components of the dystrophin-glycoprotein complex has been established by complementary, DNA cloning. The transmembrane 43K and extracellular 156K dystrophin-associated glycoproteins (DAGs) are encoded by a single messenger RNA and the extracellular 156K DAG binds laminin. Thus, the 156K DAG is a new laminin-binding glycoprotein which may provide a linkage between the sarcolemma and extracellular matrix. These results support the hypothesis that the dramatic reduction in the 156K DAG in Duchenne muscular dystrophy leads to a loss of a linkage between the sarcolemma and extracellular matrix and that this may render muscle fibres more susceptible to necrosis.
Ibraghimov-Beskrovnaya
O
O
Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242.
Ervasti
J M
JM
Leveille
C J
CJ
Slaughter
C A
CA
Sernett
S W
SW
Campbell
K P
KP
D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D003001:Cloning, Molecular; D003598:Cytoskeletal Proteins / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism; D049030:Dystroglycans; D016189:Dystrophin / Q000378:metabolism*; D005109:Extracellular Matrix / Q000378:metabolism*; D015870:Gene Expression; D006801:Humans; D015151:Immunoblotting; D007797:Laminin / Q000378:metabolism; D008562:Membrane Glycoproteins; D008969:Molecular Sequence Data; D008970:Molecular Weight; D009132:Muscles / Q000737:chemistry / Q000378:metabolism; D009136:Muscular Dystrophies / Q000235:genetics / Q000378:metabolism*; D012333:RNA, Messenger / Q000032:analysis; D011993:Recombinant Fusion Proteins; D015183:Restriction Mapping; D012508:Sarcolemma / Q000378:metabolism*; D014018:Tissue Distribution
D003598:Cytoskeletal Proteins; C483081:DAG1 protein, human; D016189:Dystrophin; D007797:Laminin; D008562:Membrane Glycoproteins; D012333:RNA, Messenger; D011993:Recombinant Fusion Proteins; D049030:Dystroglycans