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Titolo:
MURINE AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY-DISEASE WITH MULTIORGAN INVOLVEMENT INDUCED BY THE CPK GENE
Autore:
GATTONE VH; MACNAUGHTON KA; KRAYBILL AL;
Indirizzi:
UNIV KANSAS,MED CTR,DEPT ANAT & CELL BIOL,KANSAS UNIV KIDNEY & UROL RES CTR,3901 RAINBOW BLVD KANSAS CITY KS 66160
Titolo Testata:
The Anatomical record
fascicolo: 3, volume: 245, anno: 1996,
pagine: 488 - 499
SICI:
0003-276X(1996)245:3<488:MARPKW>2.0.ZU;2-7
Fonte:
ISI
Lingua:
ENG
Soggetto:
CYSTIC-DISEASE; MOUSE; MODEL; MICE; ABNORMALITIES; EXPRESSION; DUCT; RAT;
Keywords:
MOUSE; POLYCYSTIC KIDNEY DISEASE-INFANTILE; PANCREATIC CYSTS; BILIARY CYSTS; IMMUNE SYSTEM;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
38
Recensione:
Indirizzi per estratti:
Citazione:
V.H. Gattone et al., "MURINE AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY-DISEASE WITH MULTIORGAN INVOLVEMENT INDUCED BY THE CPK GENE", The Anatomical record, 245(3), 1996, pp. 488-499

Abstract

Background: Autosomal recessive (AR) polycystic kidney disease (PKD) is characterized in humans and mice as a rapidly progressive, collecting duct cystic disease usually leading to uremia in the neonatal or infantile period. In humans, ARPKD renal pathology can be variable in severity and is associated with the development of prominent bile duct and Liver pathology. The CB7BL/6J-cpk/cpk mouse model of ARPKD is the most extensively studied murine model of inherited infantile PKD; however, these mice lack extrarenal pathology. Methods: In the present study, the cpk gene was backbreed onto CD1 mice to examine the developmentof cpk-induced ARPKD in this outbred mouse background. Resulting cystic offspring were examined morphologically and their serum urea nitrogen levels were assessed. Results: The rapid development of PKD in CD1 mice homozygous for the cpk gene appears to be slightly more rapid butotherwise comparable to that seen in inbred C57BL/6J mice. In CD1-cpk/cpk mice, the principal renal pathological finding is collecting ductcysts, which are Lined by a relatively uniform epithelium. This epithelium appears to be relatively undifferentiated based on almost total absence of intercalated cells. Proximal tubule cysts are prominent in the first postnatal week while collecting duct cysts predominate in the later stages of the disease. Extrarenal manifestations of the cpk gene are evident in the CD1 strain and include cysts of pancreatic, common bile, and major hepatic ducts. Intrahepatic bile ducts also have focal dilations. Primary (thymus) and secondary (spleen) lymphoid tissues become hypoplastic as azotemia progresses. The strain-related variability in renal and liver changes in cpk-induced ARPKD may reflect the influence of other genes (possibly modifier genes) expressed in this mouse strain. In older CD1-cpk/+ mice, renal (proximal tubular) cysts and prominent liver cysts (lined by a biliary epithelium) develop, indicating that the heterozygous state (cpk/+ genotype) causes renal and hepatic pathology. Conclusions: The cpk gene, when placed on an appropriate mouse strain background, causes multiorgan disease that more closely mimics human ARPKD than when the cpk gene is expressed on the C57BL/6J strain. A gene dose effect is present as cystic pathology is present in kidney and liver of both suckling homozygous (cpk/cpk) and old heterozygous (cpk/+) mice. (C) 1996 Wiley-Liss, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/11/20 alle ore 03:33:56