MT-ND4 is translated

Stable Identifier
R-HSA-9906955
Type
Reaction [uncertain]
Species
Homo sapiens
Compartment
mitochondrial inner membrane, mitochondrial matrix
ReviewStatus
5/5
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MT-ND4 is translated
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Oxidase assembly 1-like protein (OXA1-like protein, OXA1L) catalyzes the cotranslational insertion of mitochondrial proteins in the inner mitochondrial membrane. OXA1L is in complex with TMEM126A which stabilizes OXA1L. In particular, the Complex I subunit MT-ND4 has been shown to require OXA1L and TMEM126A for assembly with human Complex I (Stiburek et al., 2007; D'Angelo et al., 2021; Formosa et al., 2021; Poerschke et al., 2024).
Literature References
PubMed ID Title Journal Year
33879611 Optic atrophy-associated TMEM126A is an assembly factor for the ND4-module of mitochondrial complex I

Formosa, LE, Reljic, B, Sharpe, AJ, Hock, DH, Muellner-Wong, L, Stroud, DA, Ryan, MT

Proc Natl Acad Sci U S A 2021
38199007 Identification of TMEM126A as OXA1L-interacting protein reveals cotranslational quality control in mitochondria

Poerschke, S, Oeljeklaus, S, Cruz-Zaragoza, LD, Schenzielorz, A, Dahal, D, Hillen, HS, Das, H, Kremer, LS, Valpadashi, A, Breuer, M, Sattmann, J, Richter-Dennerlein, R, Warscheid, B, Dennerlein, S, Rehling, P

Mol Cell 2024
33882309 NDUFS3 depletion permits complex I maturation and reveals TMEM126A/OPA7 as an assembly factor binding the ND4-module intermediate

D'Angelo, L, Astro, E, De Luise, M, Kurelac, I, Umesh-Ganesh, N, Ding, S, Fearnley, IM, Gasparre, G, Zeviani, M, Porcelli, AM, Fernandez-Vizarra, E, Iommarini, L

Cell Rep 2021
17936786 Knockdown of human Oxa1l impairs the biogenesis of F1Fo-ATP synthase and NADH:ubiquinone oxidoreductase

Stiburek, L, Fornuskova, D, Wenchich, L, Pejznochova, M, Hansikova, H, Zeman, J

J Mol Biol 2007
Participants
Input
Output
Participates
as an event of
Event Information
Go Biological Process
mitochondrial translation (0032543)
Catalyst Activity

membrane insertase activity of OXA1L:TMEM126A [mitochondrial inner membrane]

Physical Entity
OXA1L:TMEM126A [mitochondrial inner membrane] (Homo sapiens)
Activity
membrane insertase activity (GO:0032977)
Authored
Stephan, R  (2024-04-04)
Reviewed
Hill, DP  (2024-05-16)
Created
Stephan, R  (2024-04-04)
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