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. 2021 May 6;12:276. doi: 10.1186/s13287-021-02355-0

Fig. 6.

Fig. 6

Uptake of GALNS within EVs is more gradual and unaffected by M6P inhibition. Deficient fibroblasts were cultured in 2000 U GALNS (+GALNS) in the presence or absence of M6P (blue bars). This showed a strong increase in GALNS enzyme activity through the initial 24 h before declining, and this GALNS activity was inhibited by M6P. Deficient fibroblasts cultured in GALNS with an activity equal to those found in EVs (+LowGALNS) (purple bars) as well as UMSC-derived EVs (+EVs) (red bars) had a GALNS activity increase, though not significant at 24 h. Treatment of Morquio A deficient fibroblasts with UMSC-derived EVs or small amounts of GALNS showed steady slight increase up to 48 h, and this increase was not inhibited by M6P, suggesting a more gradual uptake. Deficient Morquio A fibroblasts without addition of exogenous GALNS (black bars). Error bars: mean ± SD. (*P < 0.05, ***P < 0.001, two-tailed unpaired t test with Welch’s correction)