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A new immuno-, dystrophin-deficient model, the NSG-mdx(4Cv) mouse, provides evidence for functional improvement following allogeneic satellite cell transplantation.
Stem Cells. 2013 Aug; 31(8):1611-20.SC

Abstract

Transplantation of a myogenic cell population into an immunodeficient recipient is an excellent way of assessing the in vivo muscle-generating capacity of that cell population. To facilitate both allogeneic and xenogeneic transplantations of muscle-forming cells in mice, we have developed a novel immunodeficient muscular dystrophy model, the NSG-mdx(4Cv) mouse. The IL2Rg mutation, which is linked to the Dmd gene on the X chromosome, simultaneously depletes NK cells and suppresses thymic lymphomas, issues that limit the utility of the SCID/mdx model. The NSG-mdx(4Cv) mouse presents a muscular dystrophy of similar severity to the conventional mdx mouse. We show that this animal supports robust engraftment of both pig and dog muscle mononuclear cells. The question of whether satellite cells prospectively isolated by flow cytometry can confer a functional benefit upon transplantation has been controversial. Using allogeneic Pax7-ZsGreen donors and NSG-mdx(4Cv) recipients, we demonstrate definitively that as few as 900 FACS-isolated satellite cells can provide functional regeneration in vivo, in the form of an increased mean maximal force-generation capacity in cell-transplanted muscles, compared to a sham-injected control group. These studies highlight the potency of satellite cells to improve muscle function and the utility of the NSG-mdx(4Cv) model for studies on muscle regeneration and Duchenne muscular dystrophy therapy.

Authors+Show Affiliations

Lillehei Heart Institute, University of Minnesota, Minneapolis, Minnesota, USA; Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural

Language

eng

PubMed ID

23606600

Citation

Arpke, Robert W., et al. "A New Immuno-, Dystrophin-deficient Model, the NSG-mdx(4Cv) Mouse, Provides Evidence for Functional Improvement Following Allogeneic Satellite Cell Transplantation." Stem Cells (Dayton, Ohio), vol. 31, no. 8, 2013, pp. 1611-20.
Arpke RW, Darabi R, Mader TL, et al. A new immuno-, dystrophin-deficient model, the NSG-mdx(4Cv) mouse, provides evidence for functional improvement following allogeneic satellite cell transplantation. Stem Cells. 2013;31(8):1611-20.
Arpke, R. W., Darabi, R., Mader, T. L., Zhang, Y., Toyama, A., Lonetree, C. L., Nash, N., Lowe, D. A., Perlingeiro, R. C., & Kyba, M. (2013). A new immuno-, dystrophin-deficient model, the NSG-mdx(4Cv) mouse, provides evidence for functional improvement following allogeneic satellite cell transplantation. Stem Cells (Dayton, Ohio), 31(8), 1611-20. https://doi.org/10.1002/stem.1402
Arpke RW, et al. A New Immuno-, Dystrophin-deficient Model, the NSG-mdx(4Cv) Mouse, Provides Evidence for Functional Improvement Following Allogeneic Satellite Cell Transplantation. Stem Cells. 2013;31(8):1611-20. PubMed PMID: 23606600.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A new immuno-, dystrophin-deficient model, the NSG-mdx(4Cv) mouse, provides evidence for functional improvement following allogeneic satellite cell transplantation. AU - Arpke,Robert W, AU - Darabi,Radbod, AU - Mader,Tara L, AU - Zhang,Yu, AU - Toyama,Akira, AU - Lonetree,Cara-Lin, AU - Nash,Nardina, AU - Lowe,Dawn A, AU - Perlingeiro,Rita C R, AU - Kyba,Michael, PY - 2012/11/24/received PY - 2013/03/19/accepted PY - 2013/4/23/entrez PY - 2013/4/23/pubmed PY - 2014/6/16/medline KW - Dmd KW - Muscular dystrophy KW - Satellite cells KW - Transplantation KW - mdx SP - 1611 EP - 20 JF - Stem cells (Dayton, Ohio) JO - Stem Cells VL - 31 IS - 8 N2 - Transplantation of a myogenic cell population into an immunodeficient recipient is an excellent way of assessing the in vivo muscle-generating capacity of that cell population. To facilitate both allogeneic and xenogeneic transplantations of muscle-forming cells in mice, we have developed a novel immunodeficient muscular dystrophy model, the NSG-mdx(4Cv) mouse. The IL2Rg mutation, which is linked to the Dmd gene on the X chromosome, simultaneously depletes NK cells and suppresses thymic lymphomas, issues that limit the utility of the SCID/mdx model. The NSG-mdx(4Cv) mouse presents a muscular dystrophy of similar severity to the conventional mdx mouse. We show that this animal supports robust engraftment of both pig and dog muscle mononuclear cells. The question of whether satellite cells prospectively isolated by flow cytometry can confer a functional benefit upon transplantation has been controversial. Using allogeneic Pax7-ZsGreen donors and NSG-mdx(4Cv) recipients, we demonstrate definitively that as few as 900 FACS-isolated satellite cells can provide functional regeneration in vivo, in the form of an increased mean maximal force-generation capacity in cell-transplanted muscles, compared to a sham-injected control group. These studies highlight the potency of satellite cells to improve muscle function and the utility of the NSG-mdx(4Cv) model for studies on muscle regeneration and Duchenne muscular dystrophy therapy. SN - 1549-4918 UR - https://www.unboundmedicine.com/medline/citation/23606600/A_new_immuno__dystrophin_deficient_model_the_NSG_mdx_4Cv__mouse_provides_evidence_for_functional_improvement_following_allogeneic_satellite_cell_transplantation_ L2 - https://doi.org/10.1002/stem.1402 DB - PRIME DP - Unbound Medicine ER -