Although NPHS1 was first demonstrated to be the causal gene for congenital nephrotic syndrome of Finnish type [20], subsequent studies have confirmed that it was also a causative gene or susceptibility gene for a variety of kidney diseases, such as SRNS, FSGS, minimal change disease with nephrotic syndrome (MCNS), IgA nephropathy, et al. [9, 11, 12, 21–24]. Here, NPHS1 is linked to familial nephrotic syndrome.