FIGURE

Fig. 6

ID

ZDB-FIG-161027-41

Publication

Middel et al., 2016 - Dysferlin-mediated phosphatidylserine sorting engages macrophages in sarcolemma repair

Other Figures

All Figure Page

Back to All Figure Page

Fig. 6

Arginine-rich motif is mutated in myopathy patients.

(a) Zebrafish zfWRRFK-TM-C and human hWRRFR-TM-C (red box arginine-rich motif; black box: TM domain, arrow: R2042C mutation). (b,c), hWRRFR-TM-C localized to the Z-line (z) and sarcolemma (sc) before damage (b). On damage, it accumulated at the lesion (b, arrow; c, red). In contrast, hWAAFK-TM-C (c, blue) and hWAAFA-TM-C (c, yellow) mutants did not accumulate and hWAAFR-TM-C caused significantly reduced accumulation (c, green). (d) LactC2:RFP accumulated in Dysf-KD myofibers when hWRRFR-TM-C (green) but not when mutant hWAAFK-TM-C (red). was co-expressed. (e,f) zfWRCFK-TM-C (e, green) and hWRCFR-TM-C (f, green), modelling the R2042C dysferlinopathic mutation showed significantly reduced accumulation (red). The fluorescence intensity of Dysf reporters at lesion relative to levels before damage is expressed as mean±s.d. (n≥6). Note that the control data on hWRRFR-TM-C (c,f) are identical. Similarly, zfWRRFK-TM-C data (e) are also shown in Figs 3h and 5a. Scale bar, 4 µm.

Expression Data

Expression Detail

Antibody Labeling

Phenotype Data

Phenotype Detail

Acknowledgments

This image is the copyrighted work of the attributed author or publisher, and ZFIN has permission only to display this image to its users. Additional permissions should be obtained from the applicable author or publisher of the image. Full text @ Nat. Commun.