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Abstract : Steroid-resistant nephrotic syndrome is a causative agent of end-stage kidney disease in children and young people. A genetic factor is responsible for around 30% of children with steroid resistance. In the first decades of the new century, research has revealed a role for CoQ in the regulation of gene expression, mitochondrial activity, and cell signaling. Primary CoQ10 insufficiency may be susceptible to treatment with CoQ10 supplements by omitting the biosynthetic deficiencies. The objective of present research: Analyze the association between steroid response coenzyme Q10 serum levels. HPLC evaluate serum coenzyme Q10 in total 80 patients with NS (40 SRNS and 40 SSNS) and 80 health control of Iraqi origin. Highly significant difference in COQ serum level between control and nephrotic groups (p˂0.001). This is the first research project of its kind in Iraqi origin to look the relation between serum COQ10 level and pediatric steroid resistance nephrotic syndrome .serum Q10 as diagnostic biomarker in patient with ADCK4 associated SRNS.