Rescue of utrophin, dystroglycan, sarcoglycan, and dystrobrevin
expression in mdx/CT mice. Immunoblotting of 20 μg of muscle cell
protein from 5-week-old wild-type (Wt), transgenic (CT), mdx, and
mdx/CT animals showed that mdx/CT mice express levels of α- and
β-dystroglycan, utrophin, α, β, and γ, sarcoglycan, and
dystrobrevins that are equal to or higher than those found in Wt
animals. Expression of all DAPs in mdx/CT were between −14% and +
30% of wild type levels, except for utrophin (+538%) and γ
sarcoglycan (+660%), which were increased by very large amounts.
Utrophin levels were also dramatically higher at 6 months of age. By
contrast, all DAPs were reduced in expression in mdx muscles when
compared with wild-type (−36% and −70% for α- and
β-dystroglycan, −95%, −30%, and −50% for α, β, and γ
sarcoglycan, and −78% for utrophin). Molecular weights shown are
≈400 kDa for utrophin and dystrophin. ≈160 kDa for
α-dystroglycan, 43 kDa for β-dystroglycan, 50 kDa for
α-sarcoglycan, 43 kDa for β-sarcoglycan, 35kDa for γ-sarcoglycan,
53 kDa for desmin (with 50 kDa lower band in CT and mdx/CT),
≈140–180 kDa for NCAM, and 90, 65, 44, and 22 kDa for
dystrobrevin.