COQ6 and steroid-resistant nephrotic syndrome: As steroid-resistant nephrotic syndrome (SRNS) has been linked to mutations in several genes encoding CoQ biosynthetic enzymes [52,54,57,63], a podocyte-specific Coq6 KO mouse model was generated by crossing Podocin-cre mice with Coq6loxP/loxP mice in which two loxP sites surround exon 6 in the Coq6 gene.