Supplementary Materials

Supplementary Material for:

Linker proteins restore basement membrane and correct LAMA2-related muscular dystrophy in mice

Judith R. Reinhard, Shuo Lin, Karen K. McKee, Sarina Meinen, Stephanie C. Crosson, Maurizio Sury, Samantha Hobbs, Geraldine Maier, Peter D. Yurchenco, Markus A. Rüegg*

*Corresponding author. Email: markus-a.ruegg{at}unibas.ch

Published 28 June 2017, Sci. Transl. Med. 9, eaal4649 (2017)
DOI: 10.1126/scitranslmed.aal4649

This PDF file includes:

  • Materials and Methods
  • Fig. S1. Expression of laminin-α2 and laminin-α4 in muscles of LAMA2 MD and healthy controls.
  • Fig. S2. Expression of laminin-α4 at different ages in wild-type and dyW/dyW mice.
  • Fig. S3. Expression of laminin-α5 in control and diseased muscles of humans and mice.
  • Fig. S4. Schematic representation of transgenic proteins and effect of mag on αDG binding of Lm-511.
  • Fig. S5. Expression and processing of transgenes.
  • Fig. S6. Inflammation is reduced by expression of αLNNd and mag in dyW/dyW mice.
  • Fig. S7. Muscle degeneration and regeneration in 3- and 8-week-old dyW/dyW and dyW/dyW DT mice.
  • Fig. S8. Transgenic expression of αLNNd and mag in dyW/dyW mice improves muscle histology of TA and diaphragm muscle in 8-week-old mice.
  • Fig. S9. Expression of mag and αLNNd in dyW/dyW mice improves variance of fiber diameter and collagen content in 16-week-old mice.
  • Fig. S10. Phenotype and continued presence of laminin-α4 and laminin-α5 in 2-year-old dyW/dyW DT mice.
  • Table S1. Statistical analysis of fiber size classes shown in Fig. 5D.
  • Table S2. Muscle force in isolated EDL or soleus muscle from 8- or 16-week-old mice.

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Other Supplementary Material for this manuscript includes the following:

  • Table S3. (Microsoft Excel format). Single data points and exact P values.