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. 2010 Mar 8;107(12):5569–5574. doi: 10.1073/pnas.0914960107

Fig. 2.

Fig. 2.

Chemical treatment rescues PTS2 processing in PEX1- and PEX6-deficient primary fibroblasts. (A) Recovery of 42-kDa thiolase in two PEX1-G843D homozygous cell lines. (B) Recovery of 42-kDa thiolase in PEX1-G843D vs. PEX1 null cells is shown on the left, and PEX6 missense vs. null cells are shown on the right. (C) Recovery of 28 kDa PhyH in PEX1-G843D, PEX6 missense, and PEX1 null cells. (D) Recovery of 66 kDa AGPS in PEX1-G843D cells. Lane C, control; lane 1, untreated; lane 2, 200 mM TMAO; lane 3, 10 μM AD; lane 4, 10 μM Epicholestanol; lane 5, GF109203x 3 μM; lane 6, Ro31-8220 0.5 μM; lane 7, 5% glycerol; lane 8, 100 mM betaine; lane R, RCDP1 cells that were unable to import AGPS, which highlights the absence of the band corresponding to 66 kDa AGPS. Genotypes: PEX1 null-I700fs/I700fs, PEX6 null-802_815del/802_815del, and PEX6 missense-R60Q/R812W.