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Statements

Subject Item
n2:RIV%2F00216208%3A11110%2F14%3A10283206%21RIV15-MSM-11110___
rdf:type
n8:Vysledek skos:Concept
rdfs:seeAlso
http://dx.doi.org/10.1016/j.mito.2014.02.010
dcterms: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).
dcterms:title
Mitochondrial membrane assembly of TMEM70 protein Mitochondrial membrane assembly of TMEM70 protein
skos:prefLabel
Mitochondrial membrane assembly of TMEM70 protein Mitochondrial membrane assembly of TMEM70 protein
skos:notation
RIV/00216208:11110/14:10283206!RIV15-MSM-11110___
n3:aktivita
n18:S n18:P n18:I
n3:aktivity
I, P(GAP303/11/0970), P(GB14-36804G), P(LL1204), S
n3:cisloPeriodika
February
n3:dodaniDat
n9:2015
n3:domaciTvurceVysledku
n11:6270875 n11:1045261 n11:7458363 n11:9691928 n11:7191731 n11:5787726
n3:druhVysledku
n20:J
n3:duvernostUdaju
n13:S
n3:entitaPredkladatele
n19:predkladatel
n3:idSjednocenehoVysledku
29559
n3:idVysledku
RIV/00216208:11110/14:10283206
n3:jazykVysledku
n4:eng
n3:klicovaSlova
Biogenesis; TMEM70; ATP synthase; Mitochondria
n3:klicoveSlovo
n7:TMEM70 n7:Biogenesis n7:Mitochondria n7:ATP%20synthase
n3:kodStatuVydavatele
GB - Spojené království Velké Británie a Severního Irska
n3:kontrolniKodProRIV
[A5AB90338A2F]
n3:nazevZdroje
Mitochondrion
n3:obor
n12:EB
n3:pocetDomacichTvurcuVysledku
6
n3:pocetTvurcuVysledku
11
n3:projekt
n6:LL1204 n6:GB14-36804G n6:GAP303%2F11%2F0970
n3:rokUplatneniVysledku
n9:2014
n3:svazekPeriodika
15
n3:tvurceVysledku
Vrbacký, Marek Karbanová, Vendula Zeman, Jiří Hejzlarová, Kateřina Mráček, Tomáš Gombitová, Adriána Cmarko, Dušan Kratochvílová, Hana Tesařová, Markéta Houštěk, Josef Wittig, Ilka
n3:wos
000336009600001
s:issn
1567-7249
s:numberOfPages
9
n15:doi
10.1016/j.mito.2014.02.010
n17:organizacniJednotka
11110