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Histopathological Defects in Intestine in Severe Spinal Muscular Atrophy Mice Are Improved by Systemic Antisense Oligonucleotide Treatment

Fig 1

Gross anatomical features of the bowel in SMA, control and PMO25 treated SMA mice.

(A) Images of the whole bowel from 10 day old SMA (N = 3), control (N = 3) and PMO25 treated SMA mice (N = 3). Specimens were pinned in order to display the whole bowel length. Arrows indicate proximal duodenum and arrowheads identify the cecum. (B) Representative image of SMA and PMO25 treated SMA mice at PND10. (C) SMA mice had the shortest whole bowel length compared to control and PMO25 treated mice. (P< 0.0001 vs control and vs SMA+PMO25. N = 6–7). (D) The mean small bowel length was significantly lower in SMA mice than in control and treated mice (P< 0.0001 vs control and vs SMA+PMO25 in small bowel. N = 3–4). (E) SMA mice displayed the lowest body weight compared to control and PMO25 treated mice. (P< 0.0001 vs control and P<0.0001 vs SMA+PMO25. N = 3–4). The relative total intestine length to body weight (F) and relative small intestinal length to body weight (G) in SMA mice were significantly higher than those in control and PMO25 treated mice (P<0.0001 N = 3–4). ***P < 0.001, *P<0.05.

Fig 1

doi: https://doi.org/10.1371/journal.pone.0155032.g001