In this study, we performed direct sequencing of 40 adult-onset Chinese FSGS families, 77 sporadic FSGS cases and 157 CKD families (non-FSGS) and identified a novel heterozygous variant (c.T1655C:p.V552A) in XPO5. All the patients were carrying variants, while the normal individuals were wild type. The gene discussed is XPO5; the disease is chronic kidney disease.