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CRISPR/Cas9 technology generated a C to A change at position 270 (c.270C>A) resulting in a phenylalanine to leuine substitution at amino acid 90 (p.F90L). This corresponds to the human c.270T>A (p.F90L) variant in the glycine/phenylalanine-rich domain identified in a family with a dominantly inherited distal myopathy. Additionally, two silent mutations were introduced to interrupt the protospacer adjacent motif (PAM) and crRNA sequences, preventing unwanted re-editing of the repaired template. Western blot analysis confirmed expression of protein in quadriceps femoris muscles. (J:333950)