1. Volume 73, Issue 6, Pages 716-723 (March 2008)
Angiotensin mediates renal fibrosis in the nephropathy of glycogen storage disease type Ia 
W.H. Yiu, C.-J. Pan, R.A. Ruef, W.-T. Peng, M.F. Starost, B.C. Mansfield, J.Y. Chou 
Kidney International 
Volume 73, Issue 6, Pages (March 2008)
DOI: /sj.ki
Copyright © 2008 International Society of Nephrology Terms and Conditions

2. Figure 1
Analysis of renal expression of the Ang system in GSD-Ia mice. (a) Quantification of Agt mRNA in wild-type (○) and GSD-Ia (•) mice during postnatal development using real-time RT-PCR. Results are the mean±s.e.m. Each point represents the average of 5 or more animals. **P<0.01; ***P< (b) Western blot analysis of Agt in the kidneys of 6-week-old wild-type (+/+) and GSD-Ia (-/-) mice. (c) Immunohistochemical analysis of Agt, Ang I, and Ang II in the kidneys of GSD-Ia mice. Plates shown are kidneys from 6-week-old wild-type (+/+) and GSD-Ia (-/-) mice at magnifications of × 50 (left panels) and × 200 (right panels). Representative experiments are shown.
Kidney International  , DOI: ( /sj.ki )
Copyright © 2008 International Society of Nephrology Terms and Conditions

3. Figure 2
Analysis of renal expression of AT1 in GSD-Ia mice. (a) Quantification of AT1 mRNA in wild-type (○) and GSD-Ia (•) mice during postnatal development using real-time RT-PCR. Results are the mean±s.e.m. Each point represents the average of five or more animals. *P<0.05. (b) Western blot analysis of AT1 in the kidneys of 6-week-old wild-type (+/+) and GSD-Ia (-/-) mice.
Kidney International  , DOI: ( /sj.ki )
Copyright © 2008 International Society of Nephrology Terms and Conditions

4. Figure 3
Analysis of renal expression of TGF-β1 in GSD-Ia mice. (a) Quantification of TGF-β1 mRNA in wild-type (○) and GSD-Ia (•) mice during postnatal development using real-time RT-PCR. Results are the mean±s.e.m. Each point represents the average of five or more animals. *P<0.05; **P<0.01. (b) Quantification of TGF-β1 protein levels in the kidneys of 6-week-old wild-type (+/+; n=14) and GSD-Ia (-/-; n=14) mice by immunoassays. *P<0.05. (c) Immunohistochemical analysis of TGF-β1 in the kidney of GSD-Ia mice. Plates shown are kidneys from 6-week-old wild-type (+/+) and GSD-Ia (-/-) mice at magnifications of × 50 (left panels) and × 200 (right panels). Representative experiments are shown.
Kidney International  , DOI: ( /sj.ki )
Copyright © 2008 International Society of Nephrology Terms and Conditions

5. Figure 4
Analysis of renal expression of CTGF in GSD-Ia mice. (a) Quantification of CTGF mRNA in wild-type (•) and GSD-Ia (○) mice during postnatal development using real-time RT-PCR. Results are the mean±s.e.m. Each point represents the average of five or more animals. *P<0.05; **P<0.01. (b) Western blot analysis of CTGF in the kidneys of 6-week-old wild-type (+/+) and GSD-Ia (-/-) mice. (c) Immunohistochemical analysis of CTGF in the kidney of GSD-Ia mice. Plates shown are kidneys from 6-week-old wild-type (+/+) and GSD-Ia (-/-) mice at magnifications of × 50 (left panels) and × 200 (right panels). Representative experiments are shown.
Kidney International  , DOI: ( /sj.ki )
Copyright © 2008 International Society of Nephrology Terms and Conditions

6. Figure 5
Analysis of renal expression of fibronectin and collagens in GSD-Ia mice. (a) Quantification of mRNA for fibronectin, collagens α1(I), α2(I), α1(III), and α2(IV) in wild-type (○) and GSD-Ia (•) mice during postnatal development using real-time RT-PCR. Results are the mean±s.e.m. Each point represents the average of five or more animals. *P<0.05; **P<0.01; ***P< (b and c) Immunohistochemical analysis of fibronectin (b) and collagen α2(IV) (c) deposition in the kidney of GSD-Ia mice. Plates shown are kidneys from 6-week-old wild-type (+/+) and GSD-Ia (-/-) mice at magnifications of × 50 (left panels) and × 200 (right panels). Representative experiments are shown.
Kidney International  , DOI: ( /sj.ki )
Copyright © 2008 International Society of Nephrology Terms and Conditions

7. Figure 6
The GSD-Ia mice manifest nephromegaly, hyperuricemia, and an abnormal increase in serum creatinine. (a) The weights of the kidneys relative to total body weight in wild-type (○) and GSD-Ia (•) mice during postnatal development. Each point represents the average of 5 or more animals. (b) Serum levels of uric acid in wild-type (○) and GSD-Ia (•) mice during postnatal development. Each point represents the average of eight or more animals. (c) Serum levels of creatinine in 6-week-old wild-type (+/+; n=19) and GSD-Ia (-/-; n=21) mice. Values represent mean±s.e.m. *P<0.05; **P<0.01; ***P<0.001.
Kidney International  , DOI: ( /sj.ki )
Copyright © 2008 International Society of Nephrology Terms and Conditions

8. Figure 7
Histological analysis of the kidneys in GSD-Ia mice. (a) Plates show H&E stained kidney sections in 6-week-old wild-type (+/+) and GSD-Ia (-/-) mice at magnifications of × 50 (a and c) and × 200 (b, d–f). Representative experiments are shown. (b) Masson's trichrome staining of kidney sections in 6-week-old wild-type (+/+) and GSD-Ia (-/-) mice at magnifications of × 50 (a and c) and × 200 (b, d–f). Interstatitial fibrosis is shown by the blue colored staining of the collagen fibers. The dilated tubules are denoted by asterisks.
Kidney International  , DOI: ( /sj.ki )
Copyright © 2008 International Society of Nephrology Terms and Conditions