Fig. 2

FAP accumulation and differentiation dictates the extent of adipogenic replacement of dysferlin-deficient muscle. PDGFRα staining of FAPs in a mild, moderate, and severe LGMD2B patient and non-dystrophic control muscle cross-sections; b muscles with increasing pathology from 12Mo B6A/J and c gastrocnemius muscle cross-sections from 6, 12, and 18Mo B6A/J and 12Mo WT control. Scale bar = 20 µm. Quantification (mean ± SD) of muscle area labeled with PDGFRα in d human and mouse muscle with e increasing pathology and f increasing age. n = 4 mice/group. Statistical comparisons performed via ANOVA with Holm–Sidak multiple comparisons test for all means with 12Mo TA or 12Mo WT respectively, *p < 0.05, **p < 0.01, ***p < 0.001. g Gastrocnemius muscle cross-section of 12Mo B6A/J co-labeled for PDGFRα and Perilipin-1. Scale bar = 50 µm. h Oil Red O staining of primary cell suspension isolated from hindlimb muscle of 6Mo B6A/J prior to (uninduced) or following (induced) induction for adipogenic differentiation. Scale bar = 200 µm. Inset shows a zoomed image of the box drawn in h. Oil Red O staining of sorted cells i lacking or j expressing PDGFRα at their cell surface. Scale bar = 200 µm. k FACS scatter plots showing cell surface labeling of PDGFRα in primary cell suspension prepared from mouse hindlimb muscle. The regions drawn mark the cells that express or lack cell surface PDGFRα expression