Loading...
Please wait, while we are loading the content...
Similar Documents
Targeted deletion of the mouse Mitoferrin1 gene: from anemia to protoporphyria
| Content Provider | Scilit |
|---|---|
| Author | Troadec, Marie-Berengere Warner, David Wallace, Jared Thomas, Kirk Spangrude, Gerald J. Phillips, John Khalimonchuk, Oleh Paw, Barry Ward, Diane McVey Kaplan, Jerry |
| Copyright Year | 2011 |
| Description | Mitoferrin1 is 1 of 2 homologous mitochondrial iron transporters and is required for mitochondrial iron delivery in developing erythroid cells. We show that total deletion of Mfrn1 in embryos leads to embryonic lethality. Selective deletion of Mfrn1 in adult hematopoietic tissues leads to severe anemia because of a deficit in erythroblast formation. Deletion of Mfrn1 in hepatocytes has no phenotype or biochemical effect under normal conditions. In the presence of increased porphyrin synthesis, however, deletion of Mfrn1 in hepatocytes results in a decreased ability to convert protoporphyrin IX into heme, leading to protoporphyria, cholestasis, and bridging cirrhosis. Our results show that the activity of mitoferrin1 is required to manage an increase in heme synthesis. The data also show that alterations in heme synthesis within hepatocytes can lead to protoporphyria and hepatotoxicity. |
| Related Links | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3109720/pdf |
| Ending Page | 5502 |
| Page Count | 9 |
| Starting Page | 5494 |
| DOI | 10.1182/blood-2010-11-319483 |
| Journal | Blood |
| Issue Number | 20 |
| Volume Number | 117 |
| Language | English |
| Publisher | American Society of Hematology |
| Publisher Date | 2011-05-19 |
| Access Restriction | Open |
| Subject Keyword | Research and Experimental Medicine Hematopoietic Hepatocytes Iron Adult Heme Mitochondrial Deletion Protoporphyria Mfrn1 Mitoferrin1 Journal: Blood (Vol- 131, Issue- 20) |
| Content Type | Text |
| Resource Type | Article |
