Dystrophin-related protein is localized to neuromuscular junctions of adult skeletal muscle.
Dystrophin-related protein (DRP) is an autosomal gene product with high homology to dystrophin. We have used highly specific antibodies to the unique C-terminal peptide sequences of DRP and dystrophin to examine the subcellular localization and biochemical properties of DRP in adult skeletal muscle. DRP is enriched in isolated sarcolemma from control and mdx mouse muscle, but is much less abundant than dystrophin. Immunofluorescence microscopy localized DRP almost exclusively to the neuromuscular junction region in rabbit and mouse skeletal muscle, as well as mdx mouse muscle and denervated mouse muscle. DRP is also present in normal size and abundance and localizes to the neuromuscular junction region in muscle from the dystrophic mouse model dy/dy. Thus, DRP is a junction-specific membrane cytoskeletal protein that may play an important role in the organization of the postsynaptic membrane of the neuromuscular junction.
Ohlendieck
K
K
Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242.
Ervasti
J M
JM
Matsumura
K
K
Kahl
S D
SD
Leveille
C J
CJ
Campbell
K P
KP
D000818:Animals; D015153:Blotting, Western; D015220:Calcium Channels; D002462:Cell Membrane / Q000648:ultrastructure; D016189:Dystrophin / Q000378:metabolism*; D005455:Fluorescent Antibody Technique; D051379:Mice; D008817:Mice, Mutant Strains; D009121:Muscle Denervation; D009124:Muscle Proteins / Q000276:immunology / Q000378:metabolism*; D009469:Neuromuscular Junction / Q000378:metabolism* / Q000648:ultrastructure; D011950:Receptors, Cholinergic / Q000378:metabolism; D011978:Receptors, Nicotinic / Q000378:metabolism; D019837:Ryanodine Receptor Calcium Release Channel; D000254:Sodium-Potassium-Exchanging ATPase / Q000378:metabolism; D013569:Synapses / Q000648:ultrastructure
D015220:Calcium Channels; D016189:Dystrophin; D009124:Muscle Proteins; D011950:Receptors, Cholinergic; D011978:Receptors, Nicotinic; D019837:Ryanodine Receptor Calcium Release Channel; D000254:Sodium-Potassium-Exchanging ATPase