Fig. 3: Altered expression of the ECC genes in the muscle of SBMA mice. | Nature Communications

Fig. 3: Altered expression of the ECC genes in the muscle of SBMA mice.

From: Defective excitation-contraction coupling and mitochondrial respiration precede mitochondrial Ca2+ accumulation in spinobulbar muscular atrophy skeletal muscle

Fig. 3: Altered expression of the ECC genes in the muscle of SBMA mice.The alternative text for this image may have been generated using AI.

a–c RT-PCR analysis of the transcript levels of the indicated ECC genes normalized to beta-actin in the quadriceps muscle of a 3-week-old (light green, top row), 4-week-old (green, middle row) and 8-week-old (magenta, bottom row) WT and AR100Q mice (n = 3 mice/genotype/p20-1M; Cacna1s/Atp2a1: n = 3 mice/WT/2M, n = 6 mice/AR100Q/2M; Ryr1/Atp2a2: n = 3 mice/WT/2M, n = 4 mice/AR100Q/2M; Casq1: n = 4 mice/WT/2 M, n = 4 mice/AR100Q/2M; Casq2/Pv/Sln: n = 3 mice/WT/2M, n = 3 mice/AR100Q/2M). b 8-week-old iAR100Q mice treated with doxycycline (1 g/L), (Cacna1s/Ryr1/Atp2a1/Atp2a2/Casq1: n = 3 mice/WT, n = 5 mice/iAR100Q; Casq2/Pv: n = 3 mice/WT, n = 4 mice/iAR100Q; Sln: n = 3 mice/WT, n = 2 mice/iAR100Q); c 24-week-old knock-in AR113Q mice (Cacna1s/Casq2/Pv/Sln: n = 3 mice/WT, n = 3 mice/AR113Q; Ryr1/Atp2a1/Atp2a1: n = 3 mice/WT, n = 3 mice/AR113Q; Casq1: n = 4 mice/WT, n = 3 mice/AR113Q). d Western blot analysis of the indicated ECC genes in the quadriceps muscle of 8-week-old WT and AR100Q mice (RYR1/SERCA1/CASQ1/PV: n = 3 mice/WT, n = 4 mice/AR100Q; SLN: n = 5 mice/WT, n = 4 mice/AR100Q). ECC genes were detected with specific antibodies, and calnexin (CNX) or Ponceau S were used as a loading control. The quantification (total/CNX, or SLN/Ponceau S) normalized to each control set as 1 is shown at the bottom of each panel. The graphs show the mean ± SEM; significance was tested by the two-tailed Student’s t-test. Source data are provided as a Source Data file.

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