title: Mitochondrial membrane assembly of TMEM70 protein.
abstract: Dysfunction of TMEM70 disrupts the biogenesis of ATP synthase and represents the frequent cause of autosomal recessive encephalocardiomyopathy. We used tagged forms of TMEM70 and demonstrated that it has a hairpin structure with the N- and C-termini oriented towards the mitochondrial matrix. On BN-PAGE TMEM70 was detected in multiple forms including dimers and displayed partial overlap with assembled ATP synthase. Immunoprecipitation studies confirmed mutual interactions between TMEM70 molecules but, together with immunogold electron microscopy, not direct interaction with ATP synthase subunits. This indicates that the biological function of TMEM70 in the ATP synthase biogenesis may be mediated through interaction with other protein(s).
authors.affiliation: Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine, Charles University in Prague and General University Hospital in Prague, 12108 Prague, Czech Republic; Charles University in Prague, First Faculty of Medicine, 12108 Prague, Czech Republic.
authors.forename: Hana
authors.initials: H
authors.lastname: Kratochvílová
authors.affiliation: Charles University in Prague, First Faculty of Medicine, 12108 Prague, Czech Republic; Institute of Physiology Academy of Sciences of the Czech Republic v.v.i., 14220 Prague, Czech Republic.
authors.forename: Kateřina
authors.initials: K
authors.lastname: Hejzlarová
authors.affiliation: Institute of Physiology Academy of Sciences of the Czech Republic v.v.i., 14220 Prague, Czech Republic.
authors.forename: Marek
authors.initials: M
authors.lastname: Vrbacký
authors.affiliation: Institute of Physiology Academy of Sciences of the Czech Republic v.v.i., 14220 Prague, Czech Republic.
authors.forename: Tomáš
authors.initials: T
authors.lastname: Mráček
authors.affiliation: Institute of Physiology Academy of Sciences of the Czech Republic v.v.i., 14220 Prague, Czech Republic.
authors.forename: Vendula
authors.initials: V
authors.lastname: Karbanová
authors.affiliation: Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine, Charles University in Prague and General University Hospital in Prague, 12108 Prague, Czech Republic.
authors.forename: Markéta
authors.initials: M
authors.lastname: Tesařová
authors.affiliation: Institute of Cellular Biology and Pathology, First Faculty of Medicine, Charles University in Prague, 12108 Prague, Czech Republic.
authors.forename: Adriána
authors.initials: A
authors.lastname: Gombitová
authors.affiliation: Institute of Cellular Biology and Pathology, First Faculty of Medicine, Charles University in Prague, 12108 Prague, Czech Republic.
authors.forename: Dušan
authors.initials: D
authors.lastname: Cmarko
authors.affiliation: Functional Proteomics, SFB 815 Core Unit, Faculty of Medicine, Goethe-University, 60590 Frankfurt am Main, Germany.
authors.forename: Ilka
authors.initials: I
authors.lastname: Wittig
authors.affiliation: Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine, Charles University in Prague and General University Hospital in Prague, 12108 Prague, Czech Republic.
authors.forename: Jiří
authors.initials: J
authors.lastname: Zeman
authors.affiliation: Institute of Physiology Academy of Sciences of the Czech Republic v.v.i., 14220 Prague, Czech Republic. Electronic address: houstek@biomed.cas.cz.
authors.forename: Josef
authors.initials: J
authors.lastname: Houštěk
mesh_terms: D002460:Cell Line; D006801:Humans; D047468:Immunoprecipitation; D008565:Membrane Proteins / Q000378:metabolism*; D016253:Microscopy, Immunoelectron; D051336:Mitochondrial Membranes / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000378:metabolism*; D025261:Mitochondrial Proton-Translocating ATPases / Q000378:metabolism*; D055503:Protein Multimerization
chemical_list: D008565:Membrane Proteins; D024101:Mitochondrial Proteins; C533106:TMEM70 protein, human; D025261:Mitochondrial Proton-Translocating ATPases
keywords: ATP synthase; Biogenesis; Mitochondria; TMEM70