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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | LeBleu, Valerie Sugimoto, Hikaru Mundel, Thomas M. Gerami-Naini, Behzad Finan, Elizabeth Miller, Caroline A. Gattone, Vincent H. Lu, Lingge Shield, Charles F. Folkman, Judah Kalluri, Raghu |
| Description | Country affiliation: United States Author Affiliation: LeBleu V ( Division of Matrix Biology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02215, USA.) |
| Abstract | Patients with Alport syndrome progressively lose renal function as a result of defective type IV collagen in their glomerular basement membrane. In mice lacking the alpha3 chain of type IV collagen (Col4A3 knockout mice), a model for Alport syndrome, transplantation of wild-type bone marrow repairs the renal disease. It is unknown whether cell-based therapies that do not require transplantation have similar potential. Here, infusion of wild-type bone marrow-derived cells into unconditioned, nonirradiated Col4A3 knockout mice during the late stage of disease significantly improved renal histology and function. Furthermore, transfusion of unfractionated wild-type blood into unconditioned, nonirradiated Col4A3 knockout mice improved the renal phenotype and significantly improved survival. Injection of mouse and human embryonic stem cells into Col4A3 knockout mice produced similar results. Regardless of treatment modality, the improvement in the architecture of the glomerular basement membrane is associated with de novo expression of the alpha3(IV) chain. These data provide further support for testing cell-based therapies for Alport syndrome. |
| File Format | HTM / HTML |
| ISSN | 10466673 |
| e-ISSN | 15333450 |
| DOI | 10.1681/ASN.2009010123 |
| Journal | Journal of the American Society of Nephrology |
| Issue Number | 11 |
| Volume Number | 20 |
| Language | English |
| Publisher | American Society of Nephrology |
| Publisher Date | 2009-11-01 |
| Publisher Place | United States |
| Access Restriction | Open |
| Subject Keyword | Discipline Nephrology Nephritis, Hereditary Surgery Stem Cell Transplantation Animals Autoantigens Genetics Bone Marrow Cells Collagen Type Iv Mice Mice, Inbred C57bl Mice, Knockout |
| Content Type | Text |
| Resource Type | Article |
| Subject | Nephrology |
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