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. 2023 Aug 11;30(10):2351–2363. doi: 10.1038/s41418-023-01205-1

Fig. 3. MTDPS13 patient-associated FBXL4 mutants are defective in promoting ubiquitination and degradation of BNIP3/BNIP3L.

Fig. 3

A Schematic representation of six MTDPS13-associated FBXL4 mutations. B WB analysis of the indicated proteins in WCL from 293 T cells transfected with the indicated plasmids and treated with DMSO or MG132 (20 μM) for 5 h. C Quantification of the intensity of the indicated protein in B. (n = 3). The intensity of each band was normalized to the intensity of Actin, and One-way ANOVA test in A. *p < 0.05, **p < 0.01, ***p < 0.001, ****p < 0.0001. D 293 T cells were transfected with the indicated plasmids. The WCL were prepared and subjected to co-IP with anti-FLAG antibody. The immunoprecipitates were analyzed by WB with the indicated antibodies. E, F WB analysis of the indicated proteins in vivo ubiquitination assays performed products and WCL from 293 T cells transfected with the indicated plasmids. G WB analysis of the indicated proteins in WCL from parental and FBXL4 KO H1299 cells stably overexpressing EV, FLAG-FBXL4-WT, -I205T or –S410P mutant.