Functional Analysis of jagged1 Expression

(A) Percent affected sapje fish as determined by birefringence assay at 4 dpf. Note fewer affected fish in the jagged1 injected sapje cohort. Four separate injection experiments were performed.

(B) Genotype of sapje injected fish with jagged1a and jagged1b as compared to non-injected sapje fish. In red are dystrophin-null fish with a WT phenotype, recovered by jagged1 overexpression.

(C) Immunofluorescence of jagged1a and jagged1b overexpression in the sapje fish. WT, phenotypically affected homozygous fish for the dystrophin mutation and jagged1a and jagged1b injected with normal birefringence (recovered) were stained for myosin heavy chain (MCH) and dystrophin antibodies. Note the organization of the muscle fibers in the recovered fish muscle comparable to the WT fish (n = 10) even without dystrophin. Photographs were taken at 20x magnification.

(D) Jagged1 protein levels in the muscle of cardiotoxin injured mice one, four, and seven days after injury.

(E) Jagged1 protein levels in muscle cells during in vitro muscle differentiation.

(F) Muscle cell proliferation rate, as measured by MTT, of two WT, two escaper, and two affected GRMD dogs. Error bars indicate SEM (n = 2, three replicates).