TMEM70 facilitates biogenesis of mammalian ATP synthase by promoting subunit c incorporation into the rotor structure of the enzyme
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F67985823%3A_____%2F19%3A00520774" target="_blank" >RIV/67985823:_____/19:00520774 - isvavai.cz</a>
Alternative codes found
RIV/68378050:_____/19:00520774 RIV/61388963:_____/19:00520846
Result on the web
<a href="https://doi.org/10.1096/fj.201900685RR" target="_blank" >https://doi.org/10.1096/fj.201900685RR</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1096/fj.201900685RR" target="_blank" >10.1096/fj.201900685RR</a>
Alternative languages
Result language
angličtina
Original language name
TMEM70 facilitates biogenesis of mammalian ATP synthase by promoting subunit c incorporation into the rotor structure of the enzyme
Original language description
Biogenesis of F1Fo ATP synthase depends on TMEM70 protein, localized in the inner mitochondrial membrane of higher eukaryotes. TMEM70 absence causes severe ATP synthase deficiency and leads to a neonatal mitochondrial encephalo-cardiomyopathy in humans. However, the exact biochemical function of TMEM70 remains unknown. Using TMEM70 conditional knockout in mice we show that absence of TMEM70 impairs the early stage of enzyme biogenesis by preventing incorporation of hydrophobic subunit c into rotor structure of the enzyme. This results in formation of an incomplete, pathological enzyme complex lacking Fo proton channel composed of subunits c and a. We demonstrated direct interaction between TMEM70 and subunit c and showed that overexpression of subunit c in TMEM70‐/‐ cells partially rescued TMEM70 defect. Accordingly, TMEM70 knockdown prevented subunit c accumulation otherwise observed in F1‐deficient cells. Altogether, we identified TMEM70 as specific ancillary factor for subunit c.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
10603 - Genetics and heredity (medical genetics to be 3)
Result continuities
Project
Result was created during the realization of more than one project. More information in the Projects tab.
Continuities
P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)
Others
Publication year
2019
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
FASEB Journal
ISSN
0892-6638
e-ISSN
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Volume of the periodical
33
Issue of the periodical within the volume
12
Country of publishing house
US - UNITED STATES
Number of pages
15
Pages from-to
14103-14117
UT code for WoS article
000507466100080
EID of the result in the Scopus database
2-s2.0-85076063076