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. 2017 Aug 15;7:8295. doi: 10.1038/s41598-017-07603-z

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© The Author(s) 2017

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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Mutation of the Zpr1 gene in motor neurons causes developmental defects and respiratory failure in Zpr1 ^Hb9MNΔ mice. (a) Photograph of E18.5 control and Zpr1 ^Hb9MNΔ embryos showing developmental defects in mutant embryos, including smaller size and loss of tail. Arrows indicate the absence of tail in mutant embryos. All mutant Zpr1 ^Hb9MNΔ mice were cyanotic and dead at birth (stillbirth). Scale bar is 5 mm. (b) Spinal cord sections from the thoracic region (T9-T12) of E18.5 control and Zpr1 ^Hb9MNΔ embryos were stained with monoclonal antibody against murine ZPR1 (Clone LG-D5). Zpr1 mutation in motor neurons results in reduced ZPR1 expression in spinal cord neurons of mutant embryos. Scale bar is 25 μm. (c) ZPR1 deficiency in motor neurons results in the loss spinal cord neurons. Spinal cord sections stained with antibody against choline acetyl transferase (ChAT). Scale bar is 100 μm. (d) Loss of neurons (mean ± s.e.m.; n = 3 mice/group) in different region of the spinal cords from Zpr1 ^Hb9MNΔ embryos, cervical (31.96 ± 6.74%, p = 0.009), thoracic (30.52 ± 7.38%, p = 0.014) and lumbar (64.46 ± 7.10%, p = 0.0008) regions. (e) Zpr1 mutation causes reduction in the cytoplasmic and nuclear staining of ZPR1 and (f) SMN in spinal cord neurons and show SMA-like phenotype. (g) Spinal cord motor neurons from SMA mice show phenotype similar to Zpr1 ^Hb9MNΔ mice. Arrows indicate accumulation of ZPR1 and SMN in sub-nuclear bodies in control neurons but sub-nuclear bodies missing in Zpr1 mutant and SMA mice. Dotted arcs represent the orientation of view from the anterior horn side of the spinal cord. Scale bar is 5 μm. (h) Lungs from E18.5 Zpr1 ^Hb9MNΔ embryos were smaller than control and lungs of Zpr1 ^Hb9MNΔ embryos immediately sank in water, indicating lungs were not inflated with air. Scale bar is 2.5 mm. (i) Histological analysis shows defects in morphogenesis of lungs from Zpr1 ^Hb9MNΔ embryos and lungs were collapsed. Scale bar is 200 μm.