ArticleThe American journal of pathology1997
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.
Article in The American journal of pathology, 1997. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
14 citing papers in PubMed, 36 citations in OpenAlex.
- Primary cilia: sensors of biliary homeostasis and disease.Nature reviews. Gastroenterology & hepatology · 2026Review
- Early infiltrating macrophage subtype correlates with late-stage phenotypic outcome in a mouse model of hepatorenal fibrocystic disease.Laboratory investigation; a journal of technical methods and pathology · 2021Article
- Integrin-βAmerican journal of physiology. Renal physiology · 2020Article
- Primary cilia disruption differentially affects the infiltrating and resident macrophage compartment in the liver.American journal of physiology. Gastrointestinal and liver physiology · 2018Article
- Case study: polycystic livers in a transgenic mouse line.Comparative medicine · 2014Article
- Primary cilia elongation in response to interleukin-1 mediates the inflammatory response.Cellular and molecular life sciences : CMLS · 2012Article
- Increased Na+/H+ exchanger activity on the apical surface of a cilium-deficient cortical collecting duct principal cell model of polycystic kidney disease.American journal of physiology. Cell physiology · 2012Article
- Primary cilia mediate mechanotransduction through control of ATP-induced Ca2+ signaling in compressed chondrocytes.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2012Article
- Cilia in vertebrate development and disease.Development (Cambridge, England) · 2012Review
- An essential role for dermal primary cilia in hair follicle morphogenesis.The Journal of investigative dermatology · 2009Article
- Cilia proteins control cerebellar morphogenesis by promoting expansion of the granule progenitor pool.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2007Article
- Cystin, a novel cilia-associated protein, is disrupted in the cpk mouse model of polycystic kidney disease.The Journal of clinical investigation · 2002Article
- Polaris, a protein involved in left-right axis patterning, localizes to basal bodies and cilia.Molecular biology of the cell · 2001Article
- Chlamydomonas IFT88 and its mouse homologue, polycystic kidney disease gene tg737, are required for assembly of cilia and flagella.The Journal of cell biology · 2000Article
Corrections and comments
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
Abstract
Autosomal recessive polycystic kidney disease (ARPKD) is characterized by biliary and renal lesions that produce significant morbidity and mortality. The biliary ductual 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 mutants and will permit a more detailed analysis of the clinical and cellular pathophysiology of the hepatic defect associated with this disease.
Indexed as
Identifiers
9176412PMC1858312W1881150596What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.