FIGURE 10.

Confocal analysis of GDAP1 variants indicates that TM and HD1 regions are important for regulating mitochondrial morphology when overexpressed. GFP-fusion of human GDAP1 containing the transmembrane domain was transiently expressed in HeLa cells and analyzed using confocal microscopy. The cells were fixed and stained against the mitochondrial marker TOM20 as described in the methods section. O represents control cells and G represents cells expressing recombinant GDAP1. Solid arrowhead shows mitochondria in cells expressing GDAP1 mutants, and hollow arrowheads show mitochondria in control, un-transfected cells. A, Cells transfected with GDAP1ΔαL show the distended mitochondrial phenotype. B, The distended phenotype is present in cells transfected with GDAP1ΔNT. C, Cells transfected with GDAP1ΔHD1 do not show obvious distended mitochondrial phenotype. D, GDAP1ΔTM is present throughout the cytoplasm and the mitochondrial morphology does not appear to be affected. The size bars represent 10 μm