Figure 3.

Stability and glucan binding of WT laforin, the purified CBM and DSP domain, and mutants with biphasic melting profiles

(A) Thermal stability of the individual CBM or DSP domain compared to that of full-length laforin (FL).

(B) Binding of full-length laforin, the CBM, and the DSP domain to DP7 as measured by the change in thermal stability. In (A) and (B) bar graphs represent the average of triplicate reactions ± SD.

(C) Melting curves of WT laforin and Y294N in the presence of increasing concentrations of glycogen. Curves are representative of triplicate reactions.

(D) Residues in the CBM core and CBM-DSP interface affected by missense mutations are shown in orange. Glucan is shown in green.