Table 2.
Dystrophin deficiency alters gene expression
| C57 | mdx | mdxQ | |
|---|---|---|---|
| Mitochondrial biogenesis (PGC‐1α pathway genes) | |||
| Sirt1 | 1.00 ± 0.24 | −1.69 ± 0.14 | −2.52 ± 0.07 |
| Ppargc1a (Pgc‐1α) | 1.00 ± 0.17 | −2.77 ± 0.04* | −3.23 ± 0.02*, # |
| Esrra (Errα) | 1.00 ± 0.10 | −1.96 ± 0.13 | −5.38 ± 0.05*, # |
| Nrf1 | 1.00 ± 0.11 | −1.02 ± 0.22 | −1.51 ± 0.12 |
| Nrip1 | 1.00 ± 0.14 | 1.12 ± 0.25 | −1.16 ± 0.08 |
| Tfam | 1.00 ± 0.15 | 1.44 ± 0.26 | 1.23 ± 0.30 |
| Metabolic genes | |||
| Fnip1 | 1.00 ± 0.10 | −1.88 ± 0.06* | −2.60 ± 0.04* |
| Mtor | 1.00 ± 0.13 | −1.09 ± 0.10 | −1.54 ± 0.14 |
| Prkaa1 | 1.00 ± 0.25 | −1.03 ± 0.19 | −1.15 ± 0.11 |
| Gapdh | 1.00 ± 0.16 | −2.31 ± 0.10* | −3.59 ± 0.04* |
| Pfkm | 1.00 ± 0.07 | −2.20 ± 0.03* | −3.38 ± 0.03*, # |
| Pparg | 1.00 ± 0.17 | −1.73 ± 0.15 | −1.49 ± 0.12 |
| Cs | 1.00 ± 0.11 | −2.22 ± 0.08* | −4.67 ± 0.02*, # |
| Mdh1 | 1.00 ± 0.17 | −2.54 ± 0.13* | −6.03 ± 0.04*, # |
| Atp1a2 | 1.00 ± 0.08 | −1.74 ± 0.06* | −2.73 ± 0.05*, # |
| Cybb | 1.00 ± 0.19 | 1.93 ± 0.18* | 1.62 ± 0.29* |
| Cycs | 1.00 ± 0.16 | −2.24 ± 0.06 | −2.59 ± 0.10* |
| Mb | 1.00 ± 0.18 | −2.95 ± 0.09* | −7.52 ± 0.03*, # |
| Mt‐atp6 | 1.00 ± 0.17 | −2.39 ± 0.03* | −3.68 ± 0.01*, # |
| Mt‐co1 | 1.00 ± 0.17 | −2.34 ± 0.03* | −3.25 ± 0.01* |
| Mt‐co2 | 1.00 ± 0.15 | −2.21 ± 0.10* | −4.29 ± 0.04*, # |
| Mt‐cyb | 1.00 ± 0.19 | −2.39 ± 0.03* | −3.50 ± 0.02*, # |
| Mt‐nd1 | 1.00 ± 0.15 | −2.40 ± 0.02* | −4.02 ± 0.02*, # |
| Mt‐nd4 | 1.00 ± 0.18 | −2.44 ± 0.02* | −3.51 ± 0.02*, # |
| Uqcrc1 | 1.00 ± 0.17 | −2.61 ± 0.11* | −5.70 ± 0.04*, # |
| Tfb1m | 1.00 ± 0.35 | −1.68 ± 0.09 | −2.27 ± 0.23 |
| Tfb2m | 1.00 ± 0.15 | −1.70 ± 0.08* | −2.69 ± 0.05* |
| Ucp1 | 1.00 ± 0.23 | −1.62 ± 0.26 | −1.72 ± 0.28 |
| Ucp3 | 1.00 ± 0.19 | −3.23 ± 0.13* | −5.01 ± 0.05* |
| Ak1 | 1.00 ± 0.24 | −4.53 ± 0.10* | −3.44 ± 0.12* |
| Akt1 | 1.00 ± 0.41 | −2.17 ± 0.10 | −1.94 ± 0.12 |
| Ckm | 1.00 ± 0.16 | −3.57 ± 0.07* | −7.79 ± 0.02*, # |
| Inflammation/antioxidant genes | |||
| Nfκb1 | 1.00 ± 0.16 | −1.06 ± 0.21 | −1.83 ± 0.08 |
| Il1b | 1.00 ± 0.32 | −1.69 ± 0.19 | 1.26 ± 0.47 |
| Il5 | 1.00 ± 0.54 | −1.01 ± 0.63 | −2.06 ± 0.17 |
| Il6 | 1.00 ± 0.50 | −1.48 ± 0.20 | −2.85 ± 0.07 |
| Tlr4 | 1.00 ± 0.17 | 1.41 ± 0.48 | −1.41 ± 0.14 |
| Tnf | 1.00 ± 0.37 | −1.03 ± 0.25 | −1.77 ± 0.06 |
| Traf1 | 1.00 ± 0.25 | 3.12 ± 0.69* | 1.22 ± 0.38 |
| Traf2 | 1.00 ± 0.14 | −1.18 ± 0.22 | −2.27 ± 0.05* |
| Cat | 1.00 ± 0.12 | −1.30 ± 0.11 | −1.88 ± 0.06* |
| Gpx1 | 1.00 ± 0.26 | −1.33 ± 0.09 | −1.89 ± 0.10 |
| Gpx4 | 1.00 ± 0.18 | −1.42 ± 0.17 | −2.49 ± 0.08* |
| Gsr | 1.00 ± 0.21 | −1.35 ± 0.13 | −1.45 ± 0.07 |
| Nfe2l2 | 1.00 ± 0.11 | −1.5 ± 0.07 | −2.32 ± 0.04* |
| Nos2 | 1.00 ± 0.10 | −2.44 ± 0.14* | −1.19 ± 0.20# |
| Prdx2 | 1.00 ± 0.08 | −1.45 ± 0.06* | −3.19 ± 0.02* |
| Sod1 | 1.00 ± 0.16 | −1.14 ± 0.19 | −2.07 ± 0.13* |
| Sod2 | 1.00 ± 0.09 | −2.49 ± 0.06* | −4.58 ± 0.03*, # |
| Apoptosis genes | |||
| Bax | 1.00 ± 0.16 | 1.19 ± 0.31 | −1.61 ± 0.14 |
| Bcl2 | 1.00 ± 0.20 | 1.23 ± 0.21 | 1.17 ± 0.20 |
| Bnip2 | 1.00 ± 0.19 | −1.61 ± 0.14 | −2.88 ± 0.05*, # |
| Casp3 | 1.00 ± 0.55 | 2.63 ± 0.35 | 1.08 ± 0.29 |
| Trp53 | 1.00 ± 0.15 | 1.24 ± 0.11 | −1.63 ± 0.05*, # |
| Xiap | 1.00 ± 0.10 | −1.12 ± 0.10 | −1.56 ± 0.05* |
| Muscle repair and protein turnover genes | |||
| Fbl | 1.00 ± 0.15 | 1.43 ± 0.51 | −1.74 ± 0.06# |
| Gata2 | 1.00 ± 0.11 | −1.10 ± 0.19 | −2.00 ± 0.17*, # |
| Hspa1a | 1.00 ± 0.20 | 2.17 ± 0.75 | 5.58 ± 4.04 |
| Hspa5 | 1.00 ± 0.08 | −1.04 ± 0.15 | −1.86 ± 0.09*, # |
| Hspb1 | 1.00 ± 0.19 | 1.16 ± 0.37 | −1.85 ± 0.15 |
| Mef2c | 1.00 ± 0.13 | −1.25 ± 0.09 | −1.64 ± 0.08 |
| Mstn | 1.00 ± 0.16 | −8.02 ± 0.07* | −7.02 ± 0.11* |
| Myf5 | 1.00 ± 0.18 | −1.84 ± 0.05 | −2.86 ± 0.09* |
| Myocd | 1.00 ± 0.26 | 1.25 ± 0.59 | 1.08 ± 0.45 |
| Myod1 | 1.00 ± 0.38 | −1.10 ± 0.14 | −1.40 ± 0.12 |
| Myof | 1.00 ± 0.47 | −1.58 ± 0.04 | −1.47 ± 0.07 |
| Myog | 1.00 ± 0.13 | 4.21 ± 1.01* | 1.91 ± 0.31*, # |
| Poldip2 | 1.00 ± 0.14 | −1.82 ± 0.15* | −2.87 ± 0.06* |
| Tgfb1 | 1.00 ± 0.13 | 1.17 ± 0.19 | −1.30 ± 0.10 |
| Structural and sarcomeric genes | |||
| Dag1 | 1.00 ± 0.13 | −1.19 ± 0.11 | −1.54 ± 0.07 |
| Dtna | 1.00 ± 0.17 | −1.82 ± 0.09* | −2.38 ± 0.07* |
| Dysf | 1.00 ± 0.27 | −1.54 ± 0.12 | −1.43 ± 0.13 |
| Myh1 | 1.00 ± 0.17 | −4.74 ± 0.07* | −9.20 ± 0.02* |
| Myh2 | 1.00 ± 0.12 | −1.44 ± 0.08 | −2.11 ± 0.07* |
| Myh7 | 1.00 ± 0.10 | 1.36 ± 0.04 | −1.29 ± 0.08*, # |
| Sgca | 1.00 ± 0.15 | −2.36 ± 0.10* | −3.32 ± 0.06* |
| Utrn 3’ | 1.00 ± 0.17 | −1.30 ± 0.17 | −2.35 ± 0.05*, # |
| Utrn 5’ | 1.00 ± 0.12 | 1.36 ± 0.42 | −1.81 ± 0.07*, # |
Expression of genes involved in inflammation, apoptosis, muscle repair, muscle structure, metabolism and the Pgc‐1α pathway were largely decreased in dystrophic muscle compared to healthy. Diaphragms from 14 month old animals are represented in this table. Data are shown as fold change (mean ± SEM) relative to C57. *Significantly different from C57; #significantly different from mdx. C57 (n = 5), mdx (n = 4), mdxQ (n = 5).