title: Cardiomyocyte specific deletion of Crif1 causes mitochondrial cardiomyopathy in mice.
abstract: Mitochondria are key organelles dedicated to energy production. Crif1, which interacts with the large subunit of the mitochondrial ribosome, is indispensable for the mitochondrial translation and membrane insertion of respiratory subunits. To explore the physiological function of Crif1 in the heart, Crif1(f/f) mice were crossed with Myh6-cre/Esr1 transgenic mice, which harbor cardiomyocyte-specific Cre activity in a tamoxifen-dependent manner. The tamoxifen injections were given at six weeks postnatal, and the mutant mice survived only five months due to hypertrophic heart failure. In the mutant cardiac muscles, mitochondrial mass dramatically increased, while the inner structure was altered with lack of cristae. Mutant cardiac muscles showed decreased rates of oxygen consumption and ATP production, suggesting that Crif1 plays a critical role in the maintenance of both mitochondrial structure and respiration in cardiac muscles.
authors: Department of Biological Sciences, Seoul National University, Gwanak-gu, Seoul, Republic of Korea.
authors: Juhee
authors: J
authors: Shin
authors: Seok Hong
authors: SH
authors: Lee
authors: Min-Chul
authors: MC
authors: Kwon
authors: Dong Kwon
authors: DK
authors: Yang
authors: Ha-Rim
authors: HR
authors: Seo
authors: Jaetaek
authors: J
authors: Kim
authors: Yoon-Young
authors: YY
authors: Kim
authors: Sun-Kyoung
authors: SK
authors: Im
authors: Evan Dale
authors: ED
authors: Abel
authors: Kyong-Tai
authors: KT
authors: Kim
authors: Woo Jin
authors: WJ
authors: Park
authors: Young-Yun
authors: YY
authors: Kong
mesh_terms: D000255:Adenosine Triphosphate / Q000096:biosynthesis; D000818:Animals; D009202:Cardiomyopathies / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D018797:Cell Cycle Proteins / Q000172:deficiency / Q000235:genetics*; D019069:Cell Respiration; D003433:Crosses, Genetic; D017353:Gene Deletion; D006333:Heart Failure / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D019426:Integrases / Q000235:genetics / Q000378:metabolism; D051379:Mice; D008822:Mice, Transgenic; D008929:Mitochondria, Heart / Q000235:genetics / Q000378:metabolism* / Q000648:ultrastructure; D009206:Myocardium / Q000378:metabolism / Q000473:pathology*; D032383:Myocytes, Cardiac / Q000378:metabolism* / Q000473:pathology; D010101:Oxygen Consumption; D013629:Tamoxifen
chemical_list: D018797:Cell Cycle Proteins; C527307:Crif1 protein, mouse; D013629:Tamoxifen; D000255:Adenosine Triphosphate; C045073:Cre recombinase; D019426:Integrases