The kidney demands a high metabolic energy supply for its proper function. Mitochondria are the major source of energy supply in aerobic cells. Accumulating amounts of evidence implicate that mitochondria play a central role in renal function. For instance, individuals with mutations in genes required for mitochondrial coenzyme Q10 (CoQ10) biosynthesis pathway, such as ADCK4 and COQ6, develop steroid-resistant nephrotic syndrome characterized by edema and severe proteinuria. Despite the importance, however, little is known about mechanisms by which altered mitochondrial function leads to development of renal disease. In this study, taking advantage of the optical clarity of embryos, we aim to establish the zebrafish as an in vivo system to analyze mitochondrial properties in the kidney. A previous work of others has developed a transgenic zebrafish with mitochondria targeted CFP and membrane targeted YFP under the UAS effector element [Tg(UAS:memYFP, mitoCFP)]. Crossings of this UAS effector line into Gal4 driver lines with podocin and cdh17 promoter [Tg(podo:Gal4) and Tg(cdh17:Gal4)] drove specific expression of the transgenes in glomerular podocytes and tubular epithelial cells, respectively, in the pronephros, providing an intravital imaging platform to study mitochondria. We are currently investigating spatiotemporal characteristics of mitochondria by live imaging in these transgenic zebrafish. Zebrafish mutants for adck4 and coq6 are also being generated by CRISPR/Cas9 system in order to present models for nephrotic syndrome caused by mitochondrial defects. Combinations of the reporter fish lines with these mutants would serve as a novel and easily accessible system to study relevance of mitochondria in human glomerular disease.