Transmembrane protein 70, mitochondrial

Definition:

Scaffold protein that participates in the c-ring assembly of mitochondrial ATP synthase (F(1)F(0) ATP synthase or complex V) by facilitating the membrane insertion and oligomer formation of the subunit c/ATP5MC1 through its interaction. Therefore, participates in the early stage of mitochondrial ATP synthase biogenesis and also protects subunit c/ATP5MC1 against intramitochondrial proteolysis. In addition, binds the mitochondrial proton-transporting ATP synthase complexes I and may play a role in the stability of its membrane-bound subassemblies.

References:

[1]. Kateřina Hejzlarová, et al. Expression and processing of the TMEM70 protein. Biochim Biophys Acta. 2011 Jan;1807(1):144-9. [Content Brief]

[2]. Laura Sánchez-Caballero, et al. TMEM70 functions in the assembly of complexes I and V. Biochim Biophys Acta Bioenerg. 2020 Aug 1;1861(8):148202. [Content Brief]

[3]. Alena Cízková, et al. TMEM70 mutations cause isolated ATP synthase deficiency and neonatal mitochondrial encephalocardiomyopathy. Nat Genet. 2008 Nov;40(11):1288-90. [Content Brief]

[4]. Jana Kovalčíkova, et al. TMEM70 facilitates biogenesis of mammalian ATP synthase by promoting subunit c incorporation into the rotor structure of the enzyme. FASEB J. 2019 Dec;33(12):14103-14117. [Content Brief]

[5]. Joe Carroll, et al. TMEM70 and TMEM242 help to assemble the rotor ring of human ATP synthase and interact with assembly factors for complex I. Proc Natl Acad Sci U S A. 2021 Mar 30;118(13):e2100558118. [Content Brief]

[6]. Hela Bahri, et al. TMEM70 forms oligomeric scaffolds within mitochondrial cristae promoting in situ assembly of mammalian ATP synthase proton channel. Biochim Biophys Acta Mol Cell Res. 2021 Apr;1868(4):118942. [Content Brief]

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