Thus, COQ8B gene mutations can cause primary CoQ10 deficiency and glomerular podocyte injury resulting in COQ8B glomerular nephropathy (COQ8B-GN) or nephrotic syndrome type 9 (NPHS9), which commonly manifests as a steroid-resistant nephrotic syndrome (SRNS) (1). This evidence concerns the gene COQ8B and hyperinsulinemic hypoglycemia, familial, 4.