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. Author manuscript; available in PMC: 2013 Jan 17.
Published in final edited form as: Dev Cell. 2011 Dec 29;22(1):38–51. doi: 10.1016/j.devcel.2011.11.013

Figure 5. DEFB103 inhibits innate immune response to viral infection and inhibits muscle differentiation.

Figure 5

Real time RT-PCR quantitation of innate immune responsive genes and genes involved in muscle differentiation. Values represent mean +/− SD from triplicates and are either expressed as relative to internal standard RPL13a or as percentage relative to control condition after being normalized to RPL13a. (A) Expression levels of innate immune responder IFIH1 and secreted factor DEFB103 after infection of muscle cells with lenti-GFP or lenti-DUX4-fl. (B) Expression levels of innate immune responders IFIH1 and ISG20 after infection with lenti-GFP in the presence of untreated media (control) or media supplemented with human β-defensin 3 peptide (5.0 ug/ml) (DEFB103) or conditioned media from lenti-DUX4-fl (C.M.). (C) Endogenous expression of DEFB103 in control testis and skeletal muscle tissues (Testis 1/2, Muscle 1/2), FSHD muscle biopsies (F-Muscle 3/4) and cultured FSHD and control muscle cells (F-MB and MB). (D) Upregulation of myostatin (MSTN) in myoblasts cultured in growth media supplemented with 2.5 ug/ml DEFB103. (E) Expression levels of various muscle marker genes in response to 2.5 ug/ml uM DEFB103 when added to muscle cells cultured in differentiation media. Myf6 and Desmin were included as genes that were unchanged on the arrays. (F) Cultured human muscle cells in differentiation media for 72 hr either without (a and b) or with (c and d) 2.5 μg/ml DEFB103. a and c, phase contrast showing inhibition of fusion by DEFB103. b and d, myosin heavy chain immunofluorescence (red) and DAPI nuclear stain (blue).