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. Author manuscript; available in PMC: 2021 Sep 15.
Published in final edited form as: Biochem J. 2020 Feb 14;477(3):727–745. doi: 10.1042/BCJ20190874

Figure 1. In vitro and lysosomal stability of WT TPP1.

Figure 1.

(A) Stability of enzymatic activity of mature WT TPP1 at 60°C. Data are compiled from two independent experiments conducted in duplicate. Duplicate measurements were averaged to generate a final decay curve. (B) Uptake of recombinant TPP1 by LINCL lymphoblasts. Cells were cultured with indicated concentration of proTPP1 for two days in the presence (open circle) or absence (closed circle) of 10 mM M6P before TPP1 activity was measured in cell extracts. Non-specific uptake was subtracted from total uptake in the absence of M6P to determine uptake specific to M6P receptor mediated endocytosis (closed triangle with dotted line). M6P specific uptake was fitted in Prism using a one-site specific binding model. Data are compiled from two independent experiments conducted in duplicate. (C) Activity of WT TPP1 in the lysosome after endocytosis by LINCL lymphoblasts. Data are compiled from two independent experiments conducted in duplicate. Symbols and error bars represent mean and standard deviation respectively in all cases.