Figure 6.

RNA slot-blot analysis of α-skeletal-actin and α-cardiac-actin transcripts. RNA from patient 2 and from an age-matched control was blotted, in duplicate, onto membranes. Membranes were hybridized with oligonucleotide probes complementary to the 3′ UTR of the α-skeletal-actin or α-cardiac-actin transcripts, were washed, and then were exposed to film. The α-skeletal actin:α-cardiac actin ratio was ∼4–5:1 in both the patient and the control, on the basis of densitometric analysis. Specificity of oligonucleotide labeling was confirmed by an absence of hybridization to RNA isolated from blood, and equal loading between duplicate samples was verified by reprobing with an oligonucleotide complementary to β-spectrin.