1. DHA restores peroxisome morphogenesis in AOx- and D-BP-defective fibroblasts.
DHA restores peroxisome morphogenesis in AOx- and D-BP-defective fibroblasts. (A) Fibroblasts defective in AOx (Aa–Ad) or D-BP (Ae–Ah) were cultured in the absence (Aa,Ae) or presence of 50 μM DHA for 12 hours (Ab,Af), 24 hours (Ac,Ag) or 48 hours (Ad,Ah), and then stained with an antibody to Pex14p. (B) Peroxisome abundance per cell following treatment with 50 or 150 μM DHA for the indicated times was determined as described in Materials and Methods. (C) AOx-deficient fibroblasts were cultured with 50 μM DHA for 0, 3, 6, 9, 12 and 24 hours. Peroxisome morphology was verified using an antibody to Pex14p (left panel). Total DHA levels were analyzed as described for Table 1 (right panel). Values are mol% of total DHA in control fibroblasts. (D) Control (Da,Dc) or PEX10-deficient (Db,Dd) fibroblasts were cultured in the absence (Da,Db) or presence (Dc,Dd) of 150 μM DHA for 48 hours and then stained with an antibody to Pex14p. (E) Fibroblasts from control and individual patients defective in PEX10 and PEX2 were treated with DHA as in A and peroxisome abundance determined. Insets are higher-magnification images of the boxed regions. Scale bars: 10 μm and 2 μm (insets). Data represent the means ± s.d. of three independent experiments; *P<0.05.
Akinori Itoyama et al. J Cell Sci 2012;125:
© 2012.