FIGURE

Fig. 4

ID

ZDB-FIG-260529-17

Publication

Tan et al., 2026 - Bi-allelic variants in NDUFA5 cause a mitochondriopathy with complex I deficiency

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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.

Expression Data

Expression Detail

Antibody Labeling

Phenotype Data

Phenotype Detail

Acknowledgments

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