Murine Models of Polycystic Kidney Disease.

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Murine Models of Polycystic Kidney Disease. is …
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scholarly articleQ13442814

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P356DOI10.1016/J.DDMEC.2013.10.002
P932PMC publication ID4085131
P698PubMed publication ID25013443

P50authorGregory GerminoQ55965628
P2093author name stringLuis Fernando Menezes
P2860cites workThe gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like proteinQ24292750
NEK8 mutations affect ciliary and centrosomal localization and may cause nephronophthisisQ24306642
Mutations in a novel gene, NPHP3, cause adolescent nephronophthisis, tapeto-retinal degeneration and hepatic fibrosisQ24310102
NEK1 mutations cause short-rib polydactyly syndrome type majewskiQ24324062
Mutations in PRKCSH cause isolated autosomal dominant polycystic liver diseaseQ24531988
The mammalian gene function resource: the International Knockout Mouse ConsortiumQ24631451
Mutations in a NIMA-related kinase gene, Nek1, cause pleiotropic effects including a progressive polycystic kidney disease in miceQ24648390
Pkd1 and Pkd2 are required for normal placental developmentQ27319907
Mutations in SEC63 cause autosomal dominant polycystic liver diseaseQ28260838
Design, power, and interpretation of studies in the standard murine model of ALSQ28268686
Cardiovascular, skeletal, and renal defects in mice with a targeted disruption of the Pkd1 geneQ28507278
The ion channel polycystin-2 is required for left-right axis determination in miceQ28508558
Cardiac defects and renal failure in mice with targeted mutations in Pkd2Q28510555
Developmental expression of urine concentration-associated genes and their altered expression in murine infantile-type polycystic kidney diseaseQ28586859
A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formationQ28590393
Fibrocystin/polyductin modulates renal tubular formation by regulating polycystin-2 expression and functionQ28594258
Tolvaptan in Patients with Autosomal Dominant Polycystic Kidney DiseaseQ29037884
Systematic review of the empirical evidence of study publication bias and outcome reporting biasQ29619094
Rapamycin ameliorates PKD resulting from conditional inactivation of Pkd1.Q33702386
Molecular advances in autosomal dominant polycystic kidney diseaseQ33723862
Evidence of extraordinary growth in the progressive enlargement of renal cystsQ33827400
Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver diseaseQ33979298
Network analysis of a Pkd1-mouse model of autosomal dominant polycystic kidney disease identifies HNF4α as a disease modifier.Q34499444
The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney diseaseQ34600282
The APL paradigm and the "co-clinical trial" projectQ35568084
Epitope-tagged Pkhd1 tracks the processing, secretion, and localization of fibrocystin.Q35647871
PKHD1, the polycystic kidney and hepatic disease 1 gene, encodes a novel large protein containing multiple immunoglobulin-like plexin-transcription-factor domains and parallel beta-helix 1 repeatsQ35764727
Genetic interaction studies link autosomal dominant and recessive polycystic kidney disease in a common pathway.Q36158232
Mutations of HNF-1beta inhibit epithelial morphogenesis through dysregulation of SOCS-3.Q36299972
Functional polycystin-1 dosage governs autosomal dominant polycystic kidney disease severityQ36357916
A critical developmental switch defines the kinetics of kidney cyst formation after loss of Pkd1Q36549183
Biliary and pancreatic dysgenesis in mice harboring a mutation in Pkhd1.Q36559697
Pasireotide is more effective than octreotide in reducing hepatorenal cystogenesis in rodents with polycystic kidney and liver diseases.Q36739229
Cilia at the node of mouse embryos sense fluid flow for left-right determination via Pkd2Q37011684
Defective glucose metabolism in polycystic kidney disease identifies a new therapeutic strategy.Q37092766
Renal injury is a third hit promoting rapid development of adult polycystic kidney diseaseQ37239858
Polycystic kidney disease, cilia, and planar polarity.Q37723498
A transcriptional network in polycystic kidney diseaseQ40775992
Toxic tubular injury in kidneys from Pkd1-deletion mice accelerates cystogenesis accompanied by dysregulated planar cell polarity and canonical Wnt signaling pathwaysQ42452939
Incompletely penetrant PKD1 alleles suggest a role for gene dosage in cyst initiation in polycystic kidney diseaseQ42927109
mTOR inhibitors in polycystic kidney disease.Q43004248
Everolimus in patients with autosomal dominant polycystic kidney disease.Q43004249
Sirolimus and kidney growth in autosomal dominant polycystic kidney diseaseQ43004251
Long-term rapamycin therapy in the Han:SPRD rat model of polycystic kidney disease (PKD).Q43069328
Autosomal Recessive Polycystic Kidney Disease: The Clinical Experience in North AmericaQ44503166
Rapamycin markedly slows disease progression in a rat model of polycystic kidney diseaseQ45162599
Effectiveness of vasopressin V2 receptor antagonists OPC-31260 and OPC-41061 on polycystic kidney disease development in the PCK rat.Q45281061
Gene editing: not just for translation anymoreQ46216698
Model organisms: There's more to life than rats and fliesQ46377715
Missense mutation in sterile alpha motif of novel protein SamCystin is associated with polycystic kidney disease in (cy/+) rat.Q46738223
Kidney cysts, pancreatic cysts, and biliary disease in a mouse model of autosomal recessive polycystic kidney diseaseQ46744622
Inhibition of mTOR with sirolimus slows disease progression in Han:SPRD rats with autosomal dominant polycystic kidney disease (ADPKD).Q46749984
A defect in a novel Nek-family kinase causes cystic kidney disease in the mouse and in zebrafishQ47073857
Identification and characterization of Pkhd1, the mouse orthologue of the human ARPKD geneQ48286475
A mouse model of autosomal recessive polycystic kidney disease with biliary duct and proximal tubule dilatation.Q51752480
Octreotide inhibits hepatic cystogenesis in a rodent model of polycystic liver disease by reducing cholangiocyte adenosine 3',5'-cyclic monophosphate.Q53563592
Kinesin Family Member 12 Is a Candidate Polycystic Kidney Disease Modifier in the cpk MouseQ57314438
A mouse model for cystic biliary dysgenesis in autosomal recessive polycystic kidney disease (ARPKD)Q57908850
Somatic inactivation of Pkd2 results in polycystic kidney diseaseQ74502174
Effective treatment of an orthologous model of autosomal dominant polycystic kidney diseaseQ76390332
Comparison of Pkd1-targeted mutants reveals that loss of polycystin-1 causes cystogenesis and bone defectsQ77105186
Late onset of renal and hepatic cysts in Pkd1-targeted heterozygotesQ78167116
Inhibition of renal cystic disease development and progression by a vasopressin V2 receptor antagonistQ79075547
A functional floxed allele of Pkd1 that can be conditionally inactivated in vivoQ81096232
P433issue3-4
P921main subjectmurine modelQ122890741
P304page(s)e153-e158
P577publication date2013-12-01
P1433published inDrug discovery today. Disease mechanismsQ27721894
P1476titleMurine Models of Polycystic Kidney Disease
P478volume10

Reverse relations

Q37049561Differences in the timing and magnitude of Pkd1 gene deletion determine the severity of polycystic kidney disease in an orthologous mouse model of ADPKD.cites workP2860

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