SpringerLink
Log in

Transforming growth factor-β in renal disease with glycogen storage disease I

  • Brief Report
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript

Abstract

We report a 14-year-old patient with Japanese glycogen storage disease I (GSD-I) who was found to have proteinuria. Renal biopsy revealed massive tubular atrophy and interstitial fibrosis with mononuclear cell infiltration, but the glomeruli were almost normal. The epithelial cells of tubules contained periodic acid-Schiff-positive glycogen deposits digested by diastase. In an immunohistological study, transforming growth factor (TGF)-β expression was increased in tubular epithelial cells compared with a normal control kidney specimen. These data suggest that increased TGF-β expression is involved in the pathophysiology of renal interstitial fibrosis in a patient with GSD-I.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

References

  1. Chen YT (2000) Glycogen storage diseases. In: Scriver, Beaudet, Sly, Valle (eds) The metabolic and molecular basis of inherited disease, 8th edn. McGraw-Hill, New York, p 1533

  2. Chen YT (1991) Type I glycogen storage disease: kidney involvement, pathogenesis and its treatment. Pediatr Nephrol 5:71–76

    CAS  PubMed  Google Scholar 

  3. Kambham N, Markowitz GS, Valeri AM, Lin J, D’Agati VD (2001) Obesity-related glomerulopathy: an emerging epidemic. Kidney Int 59:1498–1509

    Article  CAS  PubMed  Google Scholar 

  4. Burchell A (1992) The molecular basis of the type I glycogen storage diseases. Bioassays 14:395–400

    CAS  Google Scholar 

  5. Lee PJ, Dalton RN, Shah V, Hindmarsh PC, Leonard JV (1995) Glomerular and tubular function in glycogen storage disease. Pediatr Nephrol 9:705–710

    CAS  PubMed  Google Scholar 

  6. Nath KA (1992) Tubulointerstitial changes as a major determinant in the progression of renal damage. Am J Kidney Dis 20:1-17

    CAS  PubMed  Google Scholar 

  7. Struts F, Müller GA (1997) Renal fibrogenesis and progression. In: Neilson EG, Couser WC (eds) Immunologic renal diseases. Lipincott-Raven, Philadelphia, pp 705–726

  8. Knecht A, Fine LG, Kleinman KS, Rodemann HP, Muller GA, Wood DD, Norman JT (1991) Fibroblasts of rabbit kidney in culture. II. Paracrine stimulation of papillary fibrobrasts by PDGF. Am J Physiol 261:F292–F199

    CAS  PubMed  Google Scholar 

  9. Border WA, Noble NA (1994) Mechanisms of disease. Transforming growth factor β in tissue fibrosis. N Engl J Med 331:1286–1292

    CAS  PubMed  Google Scholar 

  10. Eddy AA, Giachelli CM, McCulloch L, Liu E (1995) Renal expression of genes that promote interstitial inflammation and fibrosis in rats with protein-overload proteinuria. Kidney Int 47:1546–1557

    CAS  PubMed  Google Scholar 

  11. Grinnell F, Ho CH (2002) Transforming growth factor β stimulates fibroblast-collagen matrix contraction by different mechanisms in mechanically loaded and unloaded matrices. Exp Cell Res 273:248–255

    Article  CAS  PubMed  Google Scholar 

  12. **de K, Nikolic-Paterson DJ, Huang XR, Sakai H, Kurokawa K, Atkins RC, Lan HY (2001) Tubular phenotypic change in progressive tubulointerstitial fibrosis in human glomerulonephritis. Am J Kidney Dis 38:761–769

    CAS  PubMed  Google Scholar 

  13. Phillips AO, Steadman R, Morrisey K, Williams JD (1997) Polarity of stimulation and secretion of transforming growth factor-beta 1 by cultured proximal tubular cells. Am J Pathol 150:1101–1111

    CAS  PubMed  Google Scholar 

  14. Border WA, Nobel NA (1997) TGF-beta in kidney fibrosis: a target for gene therapy. Kidney Int 51:1388–1396

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatrics, School of Medicine, University of Tokushima, Tokushima, Japan

    Maki Urushihara, Shoji Kagami, Michinori Ito, Koji Yasutomo, Shuji Kondo, Akiko Kitamura, Akiyoshi Takahashi & Yasuhiro Kuroda

  2. Division of Pediatrics, Tokushima Red Cross Hospital, Chuden-cho, Tokushima 773–8502, Komatsushima City, Japan

    Maki Urushihara

Authors
  1. Maki Urushihara
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Shoji Kagami
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Michinori Ito
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Koji Yasutomo
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Shuji Kondo
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Akiko Kitamura
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Akiyoshi Takahashi
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Yasuhiro Kuroda
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Maki Urushihara.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Urushihara, M., Kagami, S., Ito, M. et al. Transforming growth factor-β in renal disease with glycogen storage disease I. Pediatr Nephrol 19, 676–678 (2004). https://doi.org/10.1007/s00467-004-1456-6

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00467-004-1456-6

Keywords

Search

Navigation