{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T10:12:25Z","timestamp":1760177545172,"version":"build-2065373602"},"reference-count":21,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2020,6,10]],"date-time":"2020-06-10T00:00:00Z","timestamp":1591747200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Pulmonary failure is the main cause of morbidity and mortality in the human chromosomal instability syndrome Ataxia-telangiectasia (A-T). Major phenotypes include recurrent respiratory tract infections and bronchiectasis, aspiration, respiratory muscle abnormalities, interstitial lung disease, and pulmonary fibrosis. At present, no effective pulmonary therapy for A-T exists. Cell therapy using adipose-derived mesenchymal stromal\/stem cells (ASCs) might be a promising approach for tissue regeneration. The aim of the present project was to investigate whether ASCs migrate into the injured lung parenchyma of Atm-deficient mice as an indication of incipient tissue damage during A-T. Therefore, ASCs isolated from luciferase transgenic mice (mASCs) were intravenously transplanted into Atm-deficient and wild-type mice. Retention kinetics of the cells were monitored using in vivo bioluminescence imaging (BLI) and completed by subsequent verification using quantitative real-time polymerase chain reaction (qRT-PCR). The in vivo imaging and the qPCR results demonstrated migration accompanied by a significantly longer retention time of transplanted mASCs in the lung parenchyma of Atm-deficient mice compared to wild type mice. In conclusion, our study suggests incipient damage in the lung parenchyma of Atm-deficient mice. In addition, our data further demonstrate that a combination of luciferase-based PCR together with BLI is a pivotal tool for tracking mASCs after transplantation in models of inflammatory lung diseases such as A-T.<\/jats:p>","DOI":"10.3390\/cells9061444","type":"journal-article","created":{"date-parts":[[2020,6,15]],"date-time":"2020-06-15T12:16:57Z","timestamp":1592223417000},"page":"1444","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Tracking of Infused Mesenchymal Stem Cells in Injured Pulmonary Tissue in Atm-Deficient Mice"],"prefix":"10.3390","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8113-3312","authenticated-orcid":false,"given":"Patrick C.","family":"Baer","sequence":"first","affiliation":[{"name":"Division of Nephrology, Department of Internal Medicine III, University Hospital, Goethe-University, 60596 Frankfurt am Main, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julia","family":"Sann","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Internal Medicine III, University Hospital, Goethe-University, 60596 Frankfurt am Main, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1292-648X","authenticated-orcid":false,"given":"Ruth Pia","family":"Duecker","sequence":"additional","affiliation":[{"name":"Division for Allergy, Pneumology and Cystic Fibrosis, Department for Children and Adolescents, University Hospital, Goethe-University, 60596 Frankfurt am Main, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Evelyn","family":"Ullrich","sequence":"additional","affiliation":[{"name":"Division of Pediatric Stem Cell Transplantation and Immunology, Department for Children and Adolescents Medicine, University Hospital Frankfurt, Goethe University, 60596 Frankfurt am Main, Germany"},{"name":"Experimental Immunology, Department for Children and Adolescents Medicine, University Hospital Frankfurt, Goethe University, 60596 Frankfurt am Main, Germany"},{"name":"German Cancer Consortium (DKTK) partner site Frankfurt\/Mainz, 60596 Frankfurt am Main, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Helmut","family":"Geiger","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Internal Medicine III, University Hospital, Goethe-University, 60596 Frankfurt am Main, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4554-0265","authenticated-orcid":false,"given":"Peter","family":"Bader","sequence":"additional","affiliation":[{"name":"Division of Pediatric Stem Cell Transplantation and Immunology, Department for Children and Adolescents Medicine, University Hospital Frankfurt, Goethe University, 60596 Frankfurt am Main, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefan","family":"Zielen","sequence":"additional","affiliation":[{"name":"Division for Allergy, Pneumology and Cystic Fibrosis, Department for Children and Adolescents, University Hospital, Goethe-University, 60596 Frankfurt am Main, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ralf","family":"Schubert","sequence":"additional","affiliation":[{"name":"Division for Allergy, Pneumology and Cystic Fibrosis, Department for Children and Adolescents, University Hospital, Goethe-University, 60596 Frankfurt am Main, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,6,10]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1016\/j.jpeds.2016.01.002","article-title":"Serum Interleukin-6 Levels and Pulmonary Function in Ataxia-Telangiectasia","volume":"171","author":"Collaco","year":"2016","journal-title":"J. Pediatr."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1258","DOI":"10.1096\/fj.14-262527","article-title":"Tumor suppressor ataxia telangiectasia mutated functions downstream of TGF-\u03b21 in orchestrating profibrotic responses","volume":"29","author":"Overstreet","year":"2015","journal-title":"FASEB J."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1016\/j.redox.2017.11.006","article-title":"Oxidative stress-driven pulmonary inflammation and fibrosis in a mouse model of human ataxia-telangiectasia","volume":"14","author":"Duecker","year":"2018","journal-title":"Redox Biol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1093\/hmg\/dds448","article-title":"Bone marrow transplantation improves the outcome of Atm-deficient mice through the migration of ATM-competent cells","volume":"22","author":"Pietzner","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"812693","DOI":"10.1155\/2012\/812693","article-title":"Adipose-derived mesenchymal stromal\/stem cells: Tissue localization, characterization, and heterogeneity","volume":"2012","author":"Baer","year":"2012","journal-title":"Stem Cells Int."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2204","DOI":"10.1242\/jcs.02932","article-title":"Mesenchymal stem cells reside in virtually all post-natal organs and tissues","volume":"119","author":"Chagastelles","year":"2006","journal-title":"J. Cell Sci."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1429","DOI":"10.1517\/14712598.2013.833603","article-title":"The potential of cell-based therapy in lung diseases","volume":"13","author":"Huleihel","year":"2013","journal-title":"Expert Opin. Biol. Ther."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1111\/resp.12050","article-title":"Mesenchymal stem cells and the lung","volume":"18","author":"Sinclair","year":"2013","journal-title":"Respirology"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"2189","DOI":"10.1089\/scd.2011.0674","article-title":"Comparative analysis of paracrine factor expression in human adult mesenchymal stem cells derived from bone marrow, adipose, and dermal tissue","volume":"21","author":"Hsiao","year":"2012","journal-title":"Stem Cells Dev."},{"key":"ref_10","doi-asserted-by":"crossref","unstructured":"Schubert, R., Sann, J., Frueh, J.T., Ullrich, E., Geiger, H., and Baer, P.C. (2018). Tracking of Adipose-Derived Mesenchymal Stromal\/Stem Cells in a Model of Cisplatin-Induced Acute Kidney Injury: Comparison of Bioluminescence Imaging versus qRT-PCR. Int. J. Mol. Sci., 19.","DOI":"10.3390\/ijms19092564"},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Eickmeier, O., Kim, S.Y., Herrmann, E., D\u00f6ring, C., Duecker, R., Voss, S., Wehner, S., H\u00f6lscher, C., Pietzner, J., and Zielen, S. (2014). Altered mucosal immune response after acute lung injury in a murine model of Ataxia Telangiectasia. BMC Pulm. Med., 14.","DOI":"10.1186\/1471-2466-14-93"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"e45","DOI":"10.1093\/nar\/29.9.e45","article-title":"A new mathematical model for relative quantification in real-time RT\u2013PCR","volume":"29","author":"Pfaffl","year":"2001","journal-title":"Nucleic Acids Res."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1601","DOI":"10.1007\/s10529-006-9127-2","article-title":"Comparison of relative mRNA quantification models and the impact of RNA integrity in quantitative real-time RT-PCR","volume":"28","author":"Fleige","year":"2006","journal-title":"Biotechnol. Lett."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1002\/ppul.21277","article-title":"Evaluation and management of pulmonary disease in ataxia-telangiectasia","volume":"45","author":"Gower","year":"2010","journal-title":"Pediatr. Pulmonol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1002\/ppul.22817","article-title":"Infections of the respiratory system in patients with ataxia-telangiectasia","volume":"49","author":"Schroeder","year":"2014","journal-title":"Pediatr. Pulmonol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"560","DOI":"10.1038\/bmt.2015.334","article-title":"Low-dose irradiation prior to bone marrow transplantation results in ATM activation and increased lethality in Atm-deficient mice","volume":"51","author":"Pietzner","year":"2016","journal-title":"Bone Marrow Transplant."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"e128060","DOI":"10.1172\/jci.insight.128060","article-title":"Mesenchymal stromal cell exosomes prevent and revert experimental pulmonary fibrosis through modulation of monocyte phenotypes","volume":"4","author":"Mansouri","year":"2019","journal-title":"JCI Insight"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1002\/sctm.19-0037","article-title":"First-in-human high-cumulative-dose stem cell therapy in idiopathic pulmonary fibrosis with rapid lung function decline","volume":"9","author":"Averyanov","year":"2020","journal-title":"Stem Cells Transl. Med."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"216","DOI":"10.14336\/AD.2020.0228","article-title":"Transplantation of ACE2- Mesenchymal Stem Cells Improves the Outcome of Patients with COVID-19 Pneumonia","volume":"11","author":"Leng","year":"2020","journal-title":"Aging Dis."},{"key":"ref_20","first-page":"573","article-title":"Reduced irradiation pulmonary fibrosis and stromal cell migration in Smad3\u2212\/\u2212 marrow chimeric mice","volume":"20","author":"Epperly","year":"2006","journal-title":"In Vivo"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1986","DOI":"10.1634\/stemcells.2005-0579","article-title":"Threshold of lung injury required for the appearance of marrow-derived lung epithelia","volume":"24","author":"Herzog","year":"2006","journal-title":"Stem Cells"}],"container-title":["Cells"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4409\/9\/6\/1444\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T09:37:25Z","timestamp":1760175445000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4409\/9\/6\/1444"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,6,10]]},"references-count":21,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2020,6]]}},"alternative-id":["cells9061444"],"URL":"https:\/\/doi.org\/10.3390\/cells9061444","relation":{},"ISSN":["2073-4409"],"issn-type":[{"type":"electronic","value":"2073-4409"}],"subject":[],"published":{"date-parts":[[2020,6,10]]}}}