Quadruple immunofluoresence images of skeletal muscle biopsies from patients with the mtDNA m.3243A>G variant

Fluorescent images of 10µm transverse muscle sections from patients carrying the pathogenic m.3243A>G mtDNA variant, captured by automated scanning at 20× magnification using Zen 2011 (blue edition) software and Zeiss Axio imager MI microscope. Exposure times across all sections for each experimental batch were maintained. Muscle sections were stained using a 'Quantitative quadruple immunohistochemistry' technique, designed to capture expression profiles of proteins belonging to oxidative phosphorylation (OXPHOS) complexes. 'OXPHOS' sections were labelled with antibodies detecting subunits of mitochondrial complex I (NDUFB8) and complex IV (COX-1), a mitochondrial mass marker (mitochondrial porin; VDAC1) and laminin, followed by incubation with secondary antibodies (Alexa Fluor 488, 546, biotinylated IgG1 and 750) and subsequently with streptavidin 647. Alongside each sample, a no-primary control section (NPC; labelled only for laminin) was processed. This resource contains .iaf, .jpg and .png files from OXPHOS and NPC sections from 17 patients (P01-P17) and five controls (C01-C05). It also contains PDFs for 10 sections for which single fibre molecular genetic data are available at https://github.com/CnrLwlss/Ahmed_2022 File naming convention: b : where n=2 or 3 and refers to the batch that the samples were processed in. P01-P17 : Sections from m.3243A>G patients C01-C05 : Non-disease controls NPC : Non-primary control - sections labelled only with laminin OXPHOS : Sections labelled for OXPHOS complexes ch1 : MTCO1 (Complex IV; 488nm) ch2 : VDAC (Porin; 546nm) ch3 : NDUFB8 (Complex I; 647nm) ch4 : Laminin (750nm)