Knockout of Tmem70 alters biogenesis of ATP synthase and leads to embryonal lethality in mice (Q47979884)

7 February 2018

http://europepmc.org/abstract/MED/28173120

title

Knockout of Tmem70 alters biogenesis of ATP synthase and leads to embryonal lethality in mice (English)

1 reference

7 February 2018

http://europepmc.org/abstract/MED/28173120

author

Marek Vrbacký

1

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Kallayanee Chawengsaksophak

11 June 2020

3

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11 June 2020

Tomáš Mráček

5

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11 June 2020

Hana Nůsková

6

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11 June 2020

David Sedmera

7

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11 June 2020

author name string

Jana Kovalčíková

2

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11 June 2020

Inken M Beck

4

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11 June 2020

František Papoušek

8

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11 June 2020

František Kolář

9

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11 June 2020

Margarita Sobol

10

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11 June 2020

Pavel Hozák

11

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11 June 2020

Radislav Sedlacek

12

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11 June 2020

Josef Houštěk

13

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11 June 2020

publication date

1 November 2016

1 reference

7 February 2018

http://europepmc.org/abstract/MED/28173120

published in

Human Molecular Genetics

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11 June 2020

volume

25

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11 June 2020

issue

21

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11 June 2020

page(s)

4674-4685

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Molecular architecture of the rotary motor in ATP synthase

11 June 2020

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Prohibitins interact genetically with Atp23, a novel processing peptidase and chaperone for the F1Fo-ATP synthase.

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TMEM70 mutations cause isolated ATP synthase deficiency and neonatal mitochondrial encephalocardiomyopathy

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Mitochondrial encephalocardio-myopathy with early neonatal onset due to TMEM70 mutation

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21 January 2018

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21 January 2018

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The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants

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21 January 2018

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Two components in pathogenic mechanism of mitochondrial ATPase deficiency: energy deprivation and ROS production.

21 January 2018

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Biochemical phenotypes associated with the mitochondrial ATP6 gene mutations at nt8993.

21 January 2018

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Dimer ribbons of ATP synthase shape the inner mitochondrial membrane

21 January 2018

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Macromolecular organization of ATP synthase and complex I in whole mitochondria.

21 January 2018

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21 January 2018

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21 January 2018

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Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage

21 January 2018

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The mitochondrial SDHD gene is required for early embryogenesis, and its partial deficiency results in persistent carotid body glomus cell activation with full responsiveness to hypoxia

21 January 2018

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A mild impairment of mitochondrial electron transport has sex-specific effects on lifespan and aging in mice

21 January 2018

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NDUFS4: creation of a mouse model mimicking a Complex I disorder

21 January 2018

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In vivo correction of COX deficiency by activation of the AMPK/PGC-1α axis

21 January 2018

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Mammalian copper chaperone Cox17p has an essential role in activation of cytochrome C oxidase and embryonic development

21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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Knockdown of F1 epsilon subunit decreases mitochondrial content of ATP synthase and leads to accumulation of subunit c.

21 January 2018

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21 January 2018

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21 January 2018

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In vivo inhibition of the mitochondrial H+-ATP synthase in neurons promotes metabolic preconditioning.

21 January 2018

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21 January 2018

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ATP synthase deficiency due to TMEM70 mutation leads to ultrastructural mitochondrial degeneration and is amenable to treatment

21 January 2018

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21 January 2018

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21 January 2018

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Diminished synthesis of subunit a (ATP6) and altered function of ATP synthase and cytochrome c oxidase due to the mtDNA 2 bp microdeletion of TA at positions 9205 and 9206.

21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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TMEM70 protein — A novel ancillary factor of mammalian ATP synthase

21 January 2018

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A novel deficiency of mitochondrial ATPase of nuclear origin

21 January 2018

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21 January 2018

Identifiers

DOI

10.1093/HMG/DDW295

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28173120%20AND%20SRC:MED&resulttype=core&format=json

11 June 2020

1 reference