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Description
  • 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).
  • 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). (en)
Title
  • Mitochondrial membrane assembly of TMEM70 protein
  • Mitochondrial membrane assembly of TMEM70 protein (en)
skos:prefLabel
  • Mitochondrial membrane assembly of TMEM70 protein
  • Mitochondrial membrane assembly of TMEM70 protein (en)
skos:notation
  • RIV/00216208:11110/14:10283206!RIV15-MSM-11110___
http://linked.open...avai/riv/aktivita
http://linked.open...avai/riv/aktivity
  • I, P(GAP303/11/0970), P(GB14-36804G), P(LL1204), S
http://linked.open...iv/cisloPeriodika
  • February
http://linked.open...vai/riv/dodaniDat
http://linked.open...aciTvurceVysledku
http://linked.open.../riv/druhVysledku
http://linked.open...iv/duvernostUdaju
http://linked.open...titaPredkladatele
http://linked.open...dnocenehoVysledku
  • 29559
http://linked.open...ai/riv/idVysledku
  • RIV/00216208:11110/14:10283206
http://linked.open...riv/jazykVysledku
http://linked.open.../riv/klicovaSlova
  • Biogenesis; TMEM70; ATP synthase; Mitochondria (en)
http://linked.open.../riv/klicoveSlovo
http://linked.open...odStatuVydavatele
  • GB - Spojené království Velké Británie a Severního Irska
http://linked.open...ontrolniKodProRIV
  • [A5AB90338A2F]
http://linked.open...i/riv/nazevZdroje
  • Mitochondrion
http://linked.open...in/vavai/riv/obor
http://linked.open...ichTvurcuVysledku
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http://linked.open...vavai/riv/projekt
http://linked.open...UplatneniVysledku
http://linked.open...v/svazekPeriodika
  • 15
http://linked.open...iv/tvurceVysledku
  • Houštěk, Josef
  • Kratochvílová, Hana
  • Vrbacký, Marek
  • Hejzlarová, Kateřina
  • Tesařová, Markéta
  • Zeman, Jiří
  • Cmarko, Dušan
  • Mráček, Tomáš
  • Gombitová, Adriána
  • Karbanová, Vendula
  • Wittig, Ilka
http://linked.open...ain/vavai/riv/wos
  • 000336009600001
issn
  • 1567-7249
number of pages
http://bibframe.org/vocab/doi
  • 10.1016/j.mito.2014.02.010
http://localhost/t...ganizacniJednotka
  • 11110
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