Administration of Bone Marrow Derived Mesenchymal Stem Cells into the Liver: Potential to Rescue Pseudoxanthoma Elasticum in a Mouse Model (Abcc6 -/-)
Pseudoxanthoma elasticum (PXE) is a heritable ectopic mineralization disorder caused by loss-of-function mutations in the ABCC6 gene which is primarily expressed in the liver. There is currently no effective treatment for PXE. In this study, we characterized bone marrow derived mesenchymal stem cell...
| Publicado en: | Journal of Biomedicine & Biotechnology Vol. 2012; pp. 1 - 12 |
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| Autores principales: | , , |
| Formato: | pictorial research tables/charts tracings Journal Article |
| Publicado: |
Wiley-Blackwell
2012
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104297766&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 104297766 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 11107243 137K jtl: Journal of Biomedicine & Biotechnology issn: 11107243 maglogo: N pubinfo: dt: 2012 vid: 2012 pid: 480 pub: Wiley-Blackwell place: Malden, Massachusetts artinfo: ui: 104297766 104297766 2011906689 NLM23251082 PMC3519347 104297766 ppf: 1 ppct: 11 formats: fmt: @attributes: type: P tig: atl: Administration of Bone Marrow Derived Mesenchymal Stem Cells into the Liver: Potential to Rescue Pseudoxanthoma Elasticum in a Mouse Model (Abcc6 -/-) aug: au: Qiujie Jiang Takahagi, Shunsuke Uitto, Jouni affil: Department of Dermatology and Cutaneous Biology, Jefferson Medical College, Thomas Jefferson University, 233 S. 10th Street, Philadelphia, PA 19107, USA sug: subj: Connective Tissue Diseases Therapy Stem Cells Liver Failure Prevention and Control Cell Transplantation Methods Funding Source Animal Studies Mice Fluoroimmunoassay Flow Cytometry Descriptive Statistics Data Analysis Software Enzyme-Linked Immunosorbent Assay T-Tests In Vivo Studies In Vitro Studies ab: Pseudoxanthoma elasticum (PXE) is a heritable ectopic mineralization disorder caused by loss-of-function mutations in the ABCC6 gene which is primarily expressed in the liver. There is currently no effective treatment for PXE. In this study, we characterized bone marrow derived mesenchymal stem cells (MSCs) and evaluated their ability to contribute to liver regeneration, with the aim to rescue PXE phenotype. The MSCs, isolated from GFP-transgenic mice by magnetic cell sorting, were shown to have high potential for hepatic differentiation, with expression of Abcc6, in culture. These cells were transplanted into the livers of 4-week-old immunodeficient Abcc6-/- mice by intrasplenic injection one day after partial hepatectomy, when peak expression of the stromal cell derived factor-1 (SDF-1) in the liver was observed. Fluorescent bioimaging analyses indicated that transplanted MSCs homed into liver between day 1 and 7, and significant numbers of GFP-positive cells were confirmed in the liver by immunofluorescence. Moreover, enhanced engraftment efficiency was observed with MSCs with high expression levels of the chemokine receptor Cxcr4, a receptor for SDF-1. These data suggest that purified MSCs have the capability of differentiating into hepatic lineages relevant to PXE pathogenesis and may contribute to partial correction of the PXE phenotype. pubtype: Academic Journal doctype: pictorial research tables/charts tracings Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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