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Autosomal recessive polycystic kidney disease (ARPKD) is a pediatric genetic nephropathy caused by mutations in PKHD1, which encodes fibrocystin. The cellular basis and epithelial dynamics of cyst formation remain incompletely understood. We used lineage-tracing systems in rat and mouse to define epithelial behavior during cystogenesis. Clonal labeling revealed that renal and biliary epithelial cells undergo marked expansion as cysts form. Genetic mosaic analysis showed that individual Pkhd1-deficient cholangiocytes can generate millimeter-scale cysts within one year. Mathematical modeling demonstrated that biliary epithelial proliferation alone can account for cyst growth to millimeter size within weeks to months, depending on initial cell number and doubling time. Transcriptomic profiling of early cystic liver identified upregulated cell-cycle regulators, including CDK1. Pharmacological CDK1 inhibition attenuated fibrocystic liver disease in vivo. These findings show that epithelial cell expansion is the primary driver of cystogenesis and loss of fibrocystin activates a pro-proliferative program that can be therapeutically targeted.
Pubmed ID: 42305588
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A tool to design target-specific primers for polymerase chain reaction (PCR). It uses Primer3 to design PCR primers and then uses BLAST and global alignment algorithm to screen primers against user-selected database in order to avoid primer pairs (all combinations including forward-reverse primer pair, forward-forward as well as reverse-reverse pairs) that can cause non-specific amplifications.
View all literature mentionsAn independent, nonprofit organization focused on mammalian genetics research to advance human health. Their mission is to discover the genetic basis for preventing, treating, and curing human disease, and to enable research for the global biomedical community. Jackson Laboratory breeds and manages colonies of mice as resources for other research institutions and laboratories, along with providing software and techniques. Jackson Lab also conducts genetic research and provides educational material for various educational levels.
View all literature mentionsImaris provides range of capabilities for working with three dimensional images. Uses flexible editing and processing functions, such as interactive surface rendering and object slicing capabilities. And output to standard TIFF, Quicktime and AVI formats. Imaris accepts virtually all image formats that are used in confocal microscopy and many of those used in wide-field image acquisition.
View all literature mentionsA commercial antibody supplier which supplies primary and secondary antibodies, biochemicals, proteins, peptides, lysates, immunoassays and other kits.
View all literature mentionsSoftware package which provides tools for testing GO terms while accounting for the topology of the GO graph. Different test statistics and different methods for eliminating local similarities and dependencies between GO terms can be implemented and applied.
View all literature mentionsSoftware package for differential gene expression analysis based on the negative binomial distribution. Used for analyzing RNA-seq data for differential analysis of count data, using shrinkage estimation for dispersions and fold changes to improve stability and interpretability of estimates.
View all literature mentionsThis polyclonal targets IgG (H+L)
View all literature mentionsThis unknown targets Rabbit IgG (H+L)
View all literature mentionsThis unknown targets alpha-SMA
View all literature mentionsThis monoclonal targets AQP2
View all literature mentionsThis monoclonal targets Cytokeratin 19 antibody [EPNCIR127B]
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis monoclonal targets Cytokeratin 19
View all literature mentionsThis monoclonal targets Caspase 3, Active Form
View all literature mentionsThis polyclonal targets Calbindin-D28k
View all literature mentionsThis unknown targets Mouse IgG (H+L)
View all literature mentionsThis polyclonal targets Uromodulin
View all literature mentionsThis polyclonal targets CDK1-Specific
View all literature mentionsThis polyclonal targets Collagen Type III (N-terminal)
View all literature mentionsThis polyclonal targets Cyclin A2
View all literature mentionsThis polyclonal targets PDGFRB
View all literature mentionsThis recombinant monoclonal targets Laminin gamma 1
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis polyclonal targets HSP90
View all literature mentionsMus musculus with name STOCK Krt19tm1(cre/ERT)Ggu/J from IMSR.
View all literature mentionsMus musculus with name B6.129P2-Gt(ROSA)26Sortm1(CAG-Brainbow2.1)Cle/J from IMSR.
View all literature mentionsCell line HEK293T is a Transformed cell line with a species of origin Homo sapiens (Human)
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