CoQ deficiency could cause nephropathies (Ozaltin, 2014) and mutation in ADCK4 (CoQ8B), a protein required for stabilizing CoQ complex in podocyte, is an etiology of steroid-resistant nephrotic syndrome (SRNS or FSGS; Ashraf et al., 2013; Widmeier et al., 2020). Here, COQ8B is linked to steroid-resistant nephrotic syndrome.