Disease stage characterization of hepatorenal fibrocystic pathology in the PCK rat model of ARPKD

Stephen B. Mason, Yun Liang, Rachel M. Sinders, Caroline A. Miller, Tracy Eggleston-Gulyas, Robin Crisler-Roberts, Peter C. Harris, Vincent H. Gattone

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

The rat Pck gene is orthologous to the human PKHD1 gene responsible for autosomal recessive polycystic kidney disease (ARPKD). Both renal and hepatic fibrocystic pathology occur in ARPKD. Affected humans have a variable rate of progression, from morbidly affected infants to those surviving into adulthood. This study evaluated the PCK rat, a model of slowly progressive ARPKD. This model originated in Japan and was rederived to be offered commercially by Charles River Laboratories (Wilmington, MA). Previous studies have described the basic aspects of PCK pathology from privately held colonies. This study provides a comprehensive characterization of rats from those commercially available. Rats were bred, maintained on a 12:12 hr light/dark cycle, fed (7002 Teklad), and water provided ad libitum. Male and female rats were evaluated from 4 through 35 weeks of age with histology and serum chemistry. As the hepatorenal fibrocystic disease progressed beyond 18 weeks, the renal pathology (kidney weight, total cyst volume) and renal dysfunction (BUN and serum creatinine) tended to be more severe in males, whereas liver pathology (liver weight as % of body weight and hepatic fibrocystic volume) tended to be more severe in females. Hyperlipidemia was evident in both genders after 18 weeks. Bile secretion was increased in PCK rats compared with age-matched Sprague Dawley rats. The PCK is an increasingly used orthologous rodent model of human ARPKD. This characterization study of hepatorenal fibrocystic pathology in PCK rats should help researchers select stages of pathology to study and/or monitor disease progression during their longitudinal studies.

Original languageEnglish
Pages (from-to)1279-1288
Number of pages10
JournalAnatomical Record
Volume293
Issue number8
DOIs
StatePublished - Aug 2010

Fingerprint

Autosomal Recessive Polycystic Kidney
pathology
animal models
Pathology
rats
kidneys
Kidney
liver
Liver
serum
bile secretion
gene
histology
Weights and Measures
blood chemistry
cyst
Blood Urea Nitrogen
secretion
Photoperiod
rodent

Keywords

  • Bile
  • Cilia
  • Hyperlipidemia
  • Liver fibrosis
  • Polycystic kidney

ASJC Scopus subject areas

  • Anatomy
  • Histology
  • Ecology, Evolution, Behavior and Systematics
  • Biotechnology
  • Medicine(all)

Cite this

Disease stage characterization of hepatorenal fibrocystic pathology in the PCK rat model of ARPKD. / Mason, Stephen B.; Liang, Yun; Sinders, Rachel M.; Miller, Caroline A.; Eggleston-Gulyas, Tracy; Crisler-Roberts, Robin; Harris, Peter C.; Gattone, Vincent H.

In: Anatomical Record, Vol. 293, No. 8, 08.2010, p. 1279-1288.

Research output: Contribution to journalArticle

Mason, SB, Liang, Y, Sinders, RM, Miller, CA, Eggleston-Gulyas, T, Crisler-Roberts, R, Harris, PC & Gattone, VH 2010, 'Disease stage characterization of hepatorenal fibrocystic pathology in the PCK rat model of ARPKD', Anatomical Record, vol. 293, no. 8, pp. 1279-1288. https://doi.org/10.1002/ar.21166
Mason, Stephen B. ; Liang, Yun ; Sinders, Rachel M. ; Miller, Caroline A. ; Eggleston-Gulyas, Tracy ; Crisler-Roberts, Robin ; Harris, Peter C. ; Gattone, Vincent H. / Disease stage characterization of hepatorenal fibrocystic pathology in the PCK rat model of ARPKD. In: Anatomical Record. 2010 ; Vol. 293, No. 8. pp. 1279-1288.
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