Aug 21, 2013

Responses of proximal tubular cells to injury in congenital renal disease: fight or flight

Pediatric Nephrology : Journal of the International Pediatric Nephrology Association
Robert L ChevalierBarbara A Thornhill


Most chronic kidney disease in children results from congenital or inherited disorders, which can be studied in mouse models. Following 2 weeks of unilateral ureteral obstruction (UUO) in the adult mouse, nephron loss is due to proximal tubular mitochondrial injury and cell death. In neonatal mice, proximal tubular cell death is delayed beyond 2 weeks of complete UUO, and release of partial UUO allows remodeling of remaining nephrons. Progressive cyst expansion develops in polycystic kidney disease (PKD), a common inherited renal disorder. The polycystic kidney and fibrosis (pcy)-mutant mouse (which develops late-onset PKD) develops thinning of the glomerulotubular junction in parallel with growth of cysts in adulthood. Renal insufficiency in nephropathic cystinosis, a rare inherited renal disorder, results from progressive tubular cystine accumulation. In the Ctns knockout mouse (a model of cystinosis), proximal tubular cells become flattened, with loss of mitochondria and thickening of tubular basement membrane. In each model, persistent obstructive or metabolic stress leads ultimately to the formation of atubular glomeruli. The initial "fight" response (proximal tubular survival) switches to a "flight" response (proximal tub...Continue Reading

  • References23
  • Citations4


  • References23
  • Citations4


Mentioned in this Paper

Metabolic Process, Cellular
Mitochondrial Toxicity
Polycystic Kidney - Body Part
Kidney Failure
CTNS gene
Chronic Kidney Insufficiency
Tubular Cells
Renal Insufficiency
SpecimenType - Cyst

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