Table 1.
Protein | Expression level at mutant NMJ compared with wild type |
---|---|
Biglycan | Not present |
MuSK* | Decreased |
AChE | Unchanged (focally misaligned) |
AChR | Unchanged |
Utrophin | Unchanged |
Rapsyn | Unchanged |
α-, β-Dystroglycan | Unchanged |
α-, β-, γ-Sarcoglycan | Unchanged |
α-Dystrobrevin-1 | Unchanged |
α-Dystrobrevin-2 | Unchanged |
α-Syntrophin | Unchanged |
β1-Syntrophin | Unchanged |
β2-Syntrophin | Unchanged |
nNOS | Unchanged |
Laminin-2 | Unchanged |
Collagen VI | Unchanged |
Sections of 5-week-old quadriceps femoris from wild-type or biglycan null animals were mounted on the same slides and immunolabeled for each of the proteins indicated. To label AChRs, rh-α-bungarotoxin was applied simultaneously. The staining intensity of each protein at the neuromuscular junctions of wild-type and biglycan null animals was compared. Similar results were observed in muscles from four pairs of mutant and wild-type animals.
*Observed with three different anti-MuSK antisera; see Materials and Methods, Antibodies.