Fig 1. Col4a5 mRNA level was measured in Col4a5-R471X mice (n=3, male, 6 week-old) and the point mutation of Col4a5 has been verified by sequencing.

Abbr. HO, homozygous;? WT, wild type.

Fig 2. The results of urine (A) and blood (B) biochemical indicators in Col4a5-R471X mice (n=2 male and 6 female).?

Abbr. WT, wild type.

Fig 3. The results of urine (A) and plasma (B) biochemical indicators in 21-weeks-old Col4a5-R471X mice (n=3/group). (Data from a cooperator)?

Abbr. WT, wild type.

Fig 4. Marked glomerular changes are recognized in all (8/8) R471X mice of 23 weeks of age.

The renal cortex shows uniform glomerular distribution, with mesangial matrix proliferation and mild sclerosis (yellow arrow). Renal tubular changes include epithelial edema (blue arrow), atrophy (orange arrow), dilation (green arrow), and necrosis (black arrow). Connective tissue proliferation (light green arrow), lymphocyte infiltration (purple arrow), and occasional protein casts (gray arrow) are noted. Such lesions are entirely absent in controls of same age. Scale bar=100 μm; magnification, 200×.

Abbr. WT, wild type.

Fig 5. Effects of Ramipril (10mg/kg) on urine (A) and blood (B) biochemical indicators of Col4a5-R471X mice over a 16-17 weeks treatment period (6 weeks of age at initiation time, n=4-5 male and 4-5 female in each group).

Abbr. WT, wild type.

Fig 6. Effects of Ramipril (10mg/kg) on urine (A) and plasma (B) biochemical indicators of male and female Col4a5-R471X mice over a 16-17 weeks treatment period respectively (6 weeks of age at initiation time, n=4-5 male and 4-5 female in each group).?

Abbr. WT, wild type.

Fig 7. Body weight (A) and serum mCystain C (B) of Col4a5-R471X mice over a 17 weeks treatment period.?(6 weeks of age at initiation time, n=4-5 male and 4-5 female in each group).?

Abbr. WT, wild type.

Fig 8. Histopathology changes of Col4a5-R471X mice over a 17 weeks treatment period.?(6 weeks of age at initiation time, n=4-5 male and 4-5 female in each group). Data are presented as mean and ± SEM.

Abbr. WT, wild type.

Fig. 9 Histopathology changes of Col4a5-R471X mice over a 17 weeks treatment period. (6 weeks of age at initiation time, n=4-5 male and 4-5 female in each group). Data are presented as mean and ± SEM.

Abbr. WT, wild type. Dilation (yellow arrow); Obstruction? (blue arrow); Lymphocyte infiltration (light blue arrow); Protein casts (green arrow)); Infarction (black arrow)); necrosis (red arrow).