| First Author | Jokinen R | Year | 2010 |
| Journal | PLoS Genet | Volume | 6 |
| Issue | 10 | Pages | e1001161 |
| PubMed ID | 20976251 | Mgi Jnum | J:167541 |
| Mgi Id | MGI:4868524 | Doi | 10.1371/journal.pgen.1001161 |
| Citation | Jokinen R, et al. (2010) Gimap3 regulates tissue-specific mitochondrial DNA segregation. PLoS Genet 6(10):e1001161 |
| abstractText | Mitochondrial DNA (mtDNA) sequence variants segregate in mutation and tissue-specific manners, but the mechanisms remain unknown. The segregation pattern of pathogenic mtDNA mutations is a major determinant of the onset and severity of disease. Using a heteroplasmic mouse model, we demonstrate that Gimap3, an outer mitochondrial membrane GTPase, is a critical regulator of this process in leukocytes. Gimap3 is important for T cell development and survival, suggesting that leukocyte survival may be a key factor in the genetic regulation of mtDNA sequence variants and in modulating human mitochondrial diseases. |