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Fig. 4

ID: ZDB-IMAGE-260529-17
Publication: Tan et al., 2026 - Bi-allelic variants in NDUFA5 cause a mitochondriopathy with complex I deficiency
All Figures: Figures for Tan et al., 2026

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Figure Caption

Fig. 4 Complex I assembly studies (A) BN-PAGE and immunoblotting (IB) on mitochondria from control and F1:II-1 lymphoblastoid cell lines (LCLs) using the indicated antibodies. Subcomplex assignment is based on the published nomenclature, which includes reference to molecular weights as first reported.17 Asterisk (∗) indicates non-specific or assigned band. CII, complex II; CIII, complex III; CIV, complex IV. (B) As for (A) but utilizing a cocktail of antibodies for CI (NDUFA9), CII (SDHA), CIII (UQCRC2), and CV (ATP5A) on F2:II-1 and control (CII deficiency) fibroblast mitochondria. (C) Comparison of the OXPHOS proteome via 2D BN-PAGE of F2:II-1 and control fibroblast mitochondria. (D) As for (A) but utilizing the indicated antibodies to decorate CI containing SCs, CIV (COXIV), and CII.

Acknowledgments

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