Disease Stage Characterization of Hepatorenal Fibrocystic Pathology in the PCK Rat Model of ARPKD

Abstract

Renal cysts in a 25 week male PCK kidney. See Mason et al., on page 1279, in this issue.

MALDI imaging MS reveals candidate lipid markers of polycystic kidney disease

“…6F ). In contrast to recent disease stage characterization in the PCK rat ( 17 ), ALP, a marker for bile duct obstruction, was unchanged, together with unaffected levels of glucose (supplementary Table II). Renal function was not deteriorated in 10-week-old PCK rats, as total protein, urea, sodium, and creatinine levels in plasma were equal to the examined SDR control group (supplementary Table II).…”

“…5B ). The molecular weight of two hydrogen study, signifi cant hyperlipidemia with an increase in both serum cholesterol and triglycerides had been reported for PCK rats of about 18 weeks of age ( 17 ). Owing to their detergent properties, excessively accumulated liver bile acids are known to be cytotoxic and to cause hepatocyte damage ( 38,39 ).…”

“…In contrast to these mice, the disease gene in PCK rats is orthologous to the polycystic kidney and hepatic disease 1 gene affected in human ARPKD ( 16 ). Several characteristics of human disease are manifest in homozygous PCK rats ( 17 ), allowing for investigation of the molecular pathogenesis and testing of experimental therapeutics ( 18 ). We therefore set out to establish a workfl ow for identifi cation of candidate lipid makers that link molecular alterations in relevant tissues with analytes that could possibly be measured in body fl uids.…”

Dichloroacetate treatment accelerates the development of pathology in rodent autosomal recessive polycystic kidney disease

“…Four-week-old male and female PCK rats used in these experiments were bred at Indiana University from breeders provided by Charles Rivers Laboratories (20). All rats were fed ad libitum with 7002 6% Teklad mouse-rat diet (Harlan, Indianapolis, IN).…”

“…that causes human ARPKD and develops both renal and hepatic pathology similar to the human condition (20,34). Like in other renal cystic diseases, these rats are polyuric and polydipsic.…”