Table 2.

Glomerular ultrastructural findings in biopsies from patients with ACTN4 mutations, along with findings in biopsies exhibiting features of primary FSGS and postadaptive FSGS^a

ID No.	Podocytes							GBM					Endothelium		Mesangium
	FP Effacement	FP Irreg.	FP Actin Condens.	μ-Villi	Vacuoles	ED AGG	GRAN	Thickening	Irreg.	DC	Subepi. EDD	Subendo. EDD	Swelling	Loss of Fenestrae	Matrix Expan.	Cellular Expan.	EDD
FSA-1622	2+	2+	1+	2+	2+	1+	0	1+	1+	0	0	0	1+	1+	1+	1+	1+
FGCI-1111	2+	2+	2+	1+	3+	3+	0	0	1+	1+	1+	1+	1+	1+	1+	1+	2+
FSA-192	2+	2+	2+	2+	1+	1+	0	2+	1+	1+	0	0	1+	1+	2+	2+	1+
FGCI-111	3+	1+	3+	2+	2+	3+	2+	0	1+	1+	0	0	1+	1+	1+	1+	1+
FGCI-1112	3+	2+	2+	3+	2+	2+	0	1+	1+	2+	0	2+	2+	2+	1+	1+	1+
1FSGS-1	3+	3+	2+	3+	2+	0	0	1+	1+	2+	0	1+	2+	2+	3+b	3+b	0
1FSGS-2	3+	3+	2+	2+	1+	0	0	0	0	1+	0	0	1+	1+	2+	2+	0
1FSGS-3	2+	3+	1+	2+	2+	0	0	0	1+	1+	0	1+	1+	1+	2+	2+	2+
1FSGS-4	4+	4+	3+	3+	1+	0	0	0	0	0	0	0	0	0	1+	1+	0
2FSGS-1	1+	2+	2+	1+	0	0	0	0	0	1+	0	0	1+	1+	2+	2+	1+
2FSGS-2	1+	1+	1+	1+	1+	0	0	0	1+	1+	0	0	1+	1+	1+	1+	0
2FSGS-3	1+	1+	1+	1+	1+	0	0	1+	0	1+	0	0	2+	2+	1+	2+	0
2FSGS-4	2+	1+	1+	2+	2+	0	0	1+	2+	1+	0	0	2+	2+	2+	1+	0

^aAll findings graded on a scale of 0 (no involvement) to 4+ (severe involvement). 1FSGS, primary FSGS; 2FSGS, postadaptive FSGS; FP, foot process; ED AGG, electron-dense intracytoplasmic aggregates; GRAN, intracytoplasmic electron-dense lysosomes (reabsorption granules); DC, double contours; EDD, electron-dense deposits.

^bNo history of diabetes in this patient.