Nickan Research Institute Journal of Renal Injury Prevention 2345-2781 8 4 2019 12 01 The importance of genetic study in steroid-resistant nephrotic syndrome 271 282 10.15171/jrip.2019.51 EN Sepideh Zununi Vahed https://orcid.org/0000-0003-0179-4562 Hakimeh Moghaddas Sani https://orcid.org/0000-0003-4718-4786 Sima Rajabzadeh Ziba Nariman-Saleh-Fam https://orcid.org/0000-0001-9307-265X Mina Hejazian Fatemeh Zununi Vahed Seyed Mahdi Hosseiniyan Khatibi Mohammadali Mohajel Shoja Mohammadreza Ardalan https://orcid.org/0000-0002-6851-5460 Journal Article 10.15171/jrip.2019.51 2019 04 22 2019 08 09 Steroid-resistant nephrotic syndrome (SRNS) is a challenging clinical task. It has heterogeneous etiology and extremely variable clinical outcomes and generally progresses to end-stage renal disease (ESRD). Different gene mutations in podocyte’s slit diaphragm, mitochondria, and cytoskeleton proteins, as well as glomerular basement membrane (GBM) have been associated with SRNS. These proteins regulate the function of the glomerular filtration barrier. Advances in genetic approaches and podocytology have led to discover the SRNS-causing genes that lead to a better understanding of the drug resistance. More than 45 genetic mutations have been recognized in the hereditary form of SRNS. This review offers an update on the current knowledge of steroid resistance-causing gene mutations in podocytes. Understanding the specific genes involved in SRNS would guarantee an optimum therapeutic benefit of steroid treatment.