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Publication : Gimap3 regulates tissue-specific mitochondrial DNA segregation.

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.
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