COQ8B and steroid-resistant nephrotic syndrome: As COQ8B mutations have been implicated in steroid-resistant nephrotic syndrome (SRNS) in patients, a podocyte-specific Coq8b KO model (Nphs2-Cre;Coq8bloxP/loxP) was generated and the mice were shown to present with glomerulopathy and renal dysfunction that started at ∼4 mo (522, 534).