Autosomal recessive polycystic kidney disease in a murine model. A gross and microscopic description

V. H. Gattone, J. P. Calvet, B. D. Cowley, Andrew Evan, T. S. Shaver, K. Helmstadter, J. J. Grantham

Research output: Contribution to journalArticle

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Abstract

The C57BL/6J-cpk genetic murine model of autosomal recessive polycystic kidney disease was examined to gain insight into the pathogenesis of renal cystic disease. Fetal through 3-week-old offspring of heterozygote matings were used to study growth parameters and morphology of this genetic form of cystic disease. The kidneys were examined by light and electron microscopy and nephron segments were microdissected. Two phases of cystic disease development were morphologically identified. The first phase in fetal and newborn affected pups was characterized by proximal tubule enlargement and a general increase in the tubular mitotic index. The proximal tubules showed cytologic abnormalities along with an increased necrotic cell index. The later phase, in one through 3-week-old cystic pups, was characterized by progressive enlargement of the kidneys due mainly to cystic change of the collecting ducts and by development of azotemia. Secondary to the azotemia was a stunted body growth. Significant tubular epithelial hyperplasia was not found by mitotic index during the second phase, but an increase in collecting duct cellularity was present. Histone H4 gene expression, which is tightly coupled to DNA synthesis and thus an index of cell proliferation, showed only a minimal increase in cystic kidneys at 1, 2, and 3 weeks of age. Therefore, the degree of cell proliferation necessary to allow the observed tubular enlargement appears to be minimal.

Original languageEnglish (US)
Pages (from-to)231-238
Number of pages8
JournalLaboratory Investigation
Volume59
Issue number2
StatePublished - 1988
Externally publishedYes

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Autosomal Recessive Polycystic Kidney
Cystic Kidney Diseases
Azotemia
Mitotic Index
Cell Proliferation
Growth Disorders
Kidney
Genetic Models
Nephrons
Heterozygote
Histones
Hyperplasia
Electron Microscopy
Gene Expression
Light
DNA
Growth

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Gattone, V. H., Calvet, J. P., Cowley, B. D., Evan, A., Shaver, T. S., Helmstadter, K., & Grantham, J. J. (1988). Autosomal recessive polycystic kidney disease in a murine model. A gross and microscopic description. Laboratory Investigation, 59(2), 231-238.

Autosomal recessive polycystic kidney disease in a murine model. A gross and microscopic description. / Gattone, V. H.; Calvet, J. P.; Cowley, B. D.; Evan, Andrew; Shaver, T. S.; Helmstadter, K.; Grantham, J. J.

In: Laboratory Investigation, Vol. 59, No. 2, 1988, p. 231-238.

Research output: Contribution to journalArticle

Gattone, VH, Calvet, JP, Cowley, BD, Evan, A, Shaver, TS, Helmstadter, K & Grantham, JJ 1988, 'Autosomal recessive polycystic kidney disease in a murine model. A gross and microscopic description', Laboratory Investigation, vol. 59, no. 2, pp. 231-238.
Gattone VH, Calvet JP, Cowley BD, Evan A, Shaver TS, Helmstadter K et al. Autosomal recessive polycystic kidney disease in a murine model. A gross and microscopic description. Laboratory Investigation. 1988;59(2):231-238.
Gattone, V. H. ; Calvet, J. P. ; Cowley, B. D. ; Evan, Andrew ; Shaver, T. S. ; Helmstadter, K. ; Grantham, J. J. / Autosomal recessive polycystic kidney disease in a murine model. A gross and microscopic description. In: Laboratory Investigation. 1988 ; Vol. 59, No. 2. pp. 231-238.
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