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. 2020 Jul;1867(7):118709. doi: 10.1016/j.bbamcr.2020.118709

Fig. 1.

Fig. 1

Morphological characteristics of peroxisomes in MFF-deficient patient fibroblasts are altered. (A) Control fibroblasts (C109) and MFF-deficient patient fibroblasts [mutations Q64* [7], L62Pfs*13+R298* [6] and E153Afs*5 [6]] were processed for immunofluorescence microscopy using antibodies directed to PEX14, a peroxisomal membrane marker. Higher magnification of boxed region is shown. Arrowheads highlight potential membrane constrictions. Scale bars, 10 μm; magnification, 5 μm. (B) Electron micrographs of peroxisomes in MFF-deficient cells (MFFQ64⁎). White arrowheads highlight peroxisomal membrane tubules, black arrowheads indicate microtubules. Scale bars, 0.2 μm. (C) Confocal (Airyscan) images of peroxisomal membrane tubules (anti-PEX14) in MFFQ64⁎ cells co-stained with anti-α-tubulin. White arrowheads indicated co-localisation of peroxisomes and microtubules. Scale bars, 3 μm. (D) Measurement of peroxisomal width (nm) of bodies and tubules based on electron micrographs of MFFQ64⁎ fibroblasts [n = 33 (bodies), 79 (tubules)]. (E) Measurement of peroxisomal length (μm) from immunofluorescence images of MFFQ64⁎ patient fibroblasts (n = 392). (F) Quantification of peroxisome number based on immunofluorescence images of control (C109) and MFFQ64⁎ fibroblasts (n = 24). Data are from at least 3 independent experiments. ***, p < 0.001; two-tailed, unpaired t-test.