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Figure 6


Fig. 6. Mislocalisation of obscurin, telethonin and MURF2 in homozygous targeted cells. Cells were coimmunostained for a range of sarcomeric proteins as indicated. Obscurin is shown for cells co-stained with either {alpha}-actinin or myosin. (A) In the top two panels, arrows indicate puncta that stain positively for obscurin. (B) In the middle two panels, MURF2 is shown for cells co-stained with either the Z-disk epitope of titin (T12) or myosin. Arrows indicate puncta that stain positively for MURF2. (C) In the bottom set of panels, cells are co-stained for Z-disk titin and telethonin, and there is some colocalisation of these two proteins (arrows). Many titin dots do not localise with telethonin, however (arrowheads). In all cases, sarcomeric organisation is lacking, and only sporadic colocalisation with titin is observed even for obscurin and telethonin, both of which contain Z-disk-binding sites. Bars, 5 µm.





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