title: CRIF1 is essential for the synthesis and insertion of oxidative phosphorylation polypeptides in the mammalian mitochondrial membrane.
abstract: Although substantial progress has been made in understanding the mechanisms underlying the expression of mtDNA-encoded polypeptides, the regulatory factors involved in mitoribosome-mediated synthesis and simultaneous insertion of mitochondrial oxidative phosphorylation (OXPHOS) polypeptides into the inner membrane of mitochondria are still unclear. In the present study, disruption of the mouse Crif1 gene, which encodes a mitochondrial protein, resulted in a profound deficiency in OXPHOS caused by the disappearance of OXPHOS subunits and complexes in vivo. CRIF1 was associated with large mitoribosomal subunits that were located close to the polypeptide exit tunnel, and the elimination of CRIF1 led to both aberrant synthesis and defective insertion of mtDNA-encoded nascent OXPHOS polypeptides into the inner membrane. CRIF1 interacted with nascent OXPHOS polypeptides and molecular chaperones, e.g., Tid1. Taken together, these results suggest that CRIF1 plays a critical role in the integration of OXPHOS polypeptides into the mitochondrial membrane in mammals.
authors: Research Center for Endocrine and Metabolic Diseases, Chungnam National University School of Medicine, Daejeon 301-721, Korea.
authors: Soung Jung
authors: SJ
authors: Kim
authors: Min-chul
authors: MC
authors: Kwon
authors: Min Jeong
authors: MJ
authors: Ryu
authors: Hyo Kyun
authors: HK
authors: Chung
authors: Surendar
authors: S
authors: Tadi
authors: Yong Kyung
authors: YK
authors: Kim
authors: Jin Man
authors: JM
authors: Kim
authors: Sang Hee
authors: SH
authors: Lee
authors: Ji Hoon
authors: JH
authors: Park
authors: Gi Ryang
authors: GR
authors: Kweon
authors: Seung-Wook
authors: SW
authors: Ryu
authors: Young Suk
authors: YS
authors: Jo
authors: Chul-Ho
authors: CH
authors: Lee
authors: Hideyuki
authors: H
authors: Hatakeyama
authors: Yu-ichi
authors: Y
authors: Goto
authors: Yong-Hyeon
authors: YH
authors: Yim
authors: Jongkyeong
authors: J
authors: Chung
authors: Young-Yun
authors: YY
authors: Kong
authors: Minho
authors: M
authors: Shong
mesh_terms: D000818:Animals; D015153:Blotting, Western; D018797:Cell Cycle Proteins / Q000378:metabolism*; D002458:Cell Fractionation; D007150:Immunohistochemistry; D051379:Mice; D051336:Mitochondrial Membranes / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000378:metabolism*; D010085:Oxidative Phosphorylation; D010455:Peptides / Q000378:metabolism*
chemical_list: D018797:Cell Cycle Proteins; C527307:Crif1 protein, mouse; D024101:Mitochondrial Proteins; D010455:Peptides