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Titolo:
DIFFERENTIAL RESCUE OF THE RENAL AND HEPATIC-DISEASE IN AN AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY-DISEASE MOUSE MUTANT - A NEW MODEL TO STUDY THE LIVER LESION
Autore:
YODER BK; RICHARDS WG; SOMMARDAHL C; SWEENEY WE; MICHAUD EJ; WILKINSON JE; AVNER ED; WOYCHIK RP;
Indirizzi:
OAK RIDGE NATL LAB,DIV LIFE SCI,BEAR CREEK RD,Y-12 PLANT OAK RIDGE TN37831 OAK RIDGE NATL LAB,DIV LIFE SCI OAK RIDGE TN 37831 UNIV TENNESSEE,DEPT PATHOL KNOXVILLE TN 00000 CASE WESTERN RESERVE UNIV,DEPT PEDIAT CLEVELAND OH 44106
Titolo Testata:
The American journal of pathology
fascicolo: 6, volume: 150, anno: 1997,
pagine: 2231 - 2241
SICI:
0002-9440(1997)150:6<2231:DROTRA>2.0.ZU;2-I
Fonte:
ISI
Lingua:
ENG
Soggetto:
MURINE MODEL; PROTOONCOGENE EXPRESSION; MECKEL SYNDROME; CYSTIC-DISEASE; ANIMAL-MODELS; MESSENGER-RNA; CPK MOUSE; MUTATION; HEREDITARY; GENE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
65
Recensione:
Indirizzi per estratti:
Citazione:
B.K. Yoder et al., "DIFFERENTIAL RESCUE OF THE RENAL AND HEPATIC-DISEASE IN AN AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY-DISEASE MOUSE MUTANT - A NEW MODEL TO STUDY THE LIVER LESION", The American journal of pathology, 150(6), 1997, pp. 2231-2241

Abstract

Autosomal recessive polycystic kidney disease (ARPKD) is characterized by biliary and renal lesions that produce significant morbidity and mortality. The biliary ductal ectasia and hepatic portal fibrosis associated with ARPKD have not been well studied even though such lesions markedly affect the clinical course of patients after renal replacement therapy such as dialysis or transplantation, Here we describe the generation of a new mouse model to study the hepatic lesions associated with polycystic kidney disease This model was generated by differentially rescuing the renal pathology in the orpk mutant mouse that displays a hepatorenal pathology that is similar to that seen in human patients with ARPKD. This was accomplished by expressing, as a transgene in the mutant animals, the cloned wild-type version of the gene associated with the mutant locus in this line of mice. Although renal function in the rescue animals is normal, the liver still exhibits biliary and ductular hyperplasia along with varying degrees of hepatic portal fibrosis that is indistinguishable from that in the mutant animals. Most important, the rescue animals survive significantly longer than mutantsand will permit a more detailed analysis of the clinical and cellularpathophysiology of the hepatic defect associated with this disease.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 20/09/20 alle ore 04:24:42