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Segment-specific c-ErbB2 expression in human autosomal recessive polycystic kidney disease
Koichi Nakanishi1, William E Sweeney1, Ellis D Avner1
1Rainbow Center for Childhood PKD, Department of Pediatrics, Rainbow Babies and Children's Hospital and Case Western Reserve University, Cleveland, Ohio.
Journal of the American Society of Nephrology : JASN
|February 7, 2001
Summary
c-ErbB2 expression increases with gestational age in autosomal recessive polycystic kidney disease (ARPKD). This heightened expression in collecting tubule cells correlates with cyst development in ARPKD kidneys.
Area of Science:
- Nephrology
- Molecular Biology
- Developmental Biology
Background:
- c-ErbB2 (HER2) is a tyrosine kinase receptor structurally related to EGFR.
- It plays roles in epithelial cell differentiation and proliferation.
- EGFR is implicated in cystic renal epithelial hyperplasia.
Purpose of the Study:
- To examine the expression pattern of c-ErbB2 in human autosomal recessive polycystic kidney disease (ARPKD).
- To correlate c-ErbB2 expression with disease progression and developmental stage in ARPKD kidneys.
Main Methods:
- Analysis of nine ARPKD kidney specimens (gestational age 17 wk to postnatal age 4 wk).
- Immunohistochemical staining for c-ErbB2 expression.
- Comparison with normal human fetal and postnatal kidneys.
Main Results:
- c-ErbB2 expression was significantly increased in ARPKD kidneys compared to controls.
- Expression levels increased with advancing gestational age.
- Increased c-ErbB2 was localized to the apical surfaces of cystic collecting tubule cells, mirroring EGFR patterns.
Conclusions:
- c-ErbB2 expression is upregulated in ARPKD, particularly in collecting duct cysts.
- The developmental-stage-specific increase suggests a role in cystogenesis and growth in ARPKD.
- Findings highlight potential therapeutic targets related to c-ErbB2 signaling in ARPKD.