A kit of antibodies, one against each of the 5 OXPHOS complexes and PDH. MitoProfile® Kit MS602a contains 220 µg of mAbs, each provided in 50 µl aliquots at a 200x working concentration. The individual mAbs were chosen because they are against a subunit labile when its complex is not assembled and because they work well in immunocytochemistry. The mAbs are against CI-NDUFB4, CII-30 kDa, CIII-Core 2 protein, CIV-subunit I, CV-OSCP and PDH E1α subunit respectively. (CI-NUDFB4 was previously listed as likely CI-NDUFA6).

Kit MS602a contains the same mabs as in MS602 but also provides mAbs against porin (60 µg) and CVα subunit (120 µg) as controls. Thus while the mAbs to Complexes I-IV and against OSCP of Complex V are type IgG1 or IgG2a, the two control mAbs are type IgG2b. Hence, it is possible to perform 2-dye immunocytochemistry as described in detail under protocols, and as shown for case studies of patients with mitochondrial disease. Kit MS602a provides appropriate secondary mabs for the 2-dye protocol i.e. anti-mouse IgG1-FITC (80 µg) anti-mouse IgG2a-FITC (40 µg) and anti-mouse IgG2b-TXRD (120 µg).

The OXPHOS immunocytochemistry kit allows rapid screening of cells or tissues for defective assembly of the OXPHOS and PDH complexes. It requires very few cells and only needle biopsy amounts of tissue.