منابع مشابه
Polarity and renal cystogenesis.
of metanephric mesenchyme in the developing kidney regulated by Wnt-4. Nature 372: 679–683, 1994 3. Yu J, Carroll TJ, Rajagopal J, Kobayashi A, Ren Q, McMahon AP: A Wnt7b-dependent pathway regulates the orientation of epithelial cell division and establishes the cortico-medullary axis of the mammalian kidney. Development 136: 161–171, 2009 4. Kuure S, Popsueva A, JakobsonM, Sainio K, Sariola H:...
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Clinical trials are underway for the treatment of tuberous sclerosis (TSC)-associated tumours using mTOR inhibitors. Here, we show that many of the earliest renal lesions from Tsc1+/- and Tsc2+/- mice do not exhibit mTOR activation, suggesting that pharmacological targeting of an alternative pathway may be necessary to prevent tumour formation. Patients with TSC often develop renal cysts and th...
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Mutations inactivating the cilia-localized Pkd1 protein result in autosomal dominant polycystic kidney disease (ADPKD), a serious inherited syndrome affecting ∼ 1 in 500 people, in which accumulation of renal cysts eventually destroys kidney function. Severity of ADPKD varies throughout the population, for reasons thought to involve differences both in intragenic Pkd1 mutations and in modifier ...
متن کاملSix2creFrs2α knockout mice are a novel model of renal cystogenesis
Six2cre-mediated deletion of Frs2α (Six2creFrs2αKO), a major fibroblast growth factor receptor (Fgfr) docking protein in mouse nephron progenitors results in perinatal renal hypoplasia; however, postnatal Six2creFrs2αKO kidneys develop cysts. We sought to determine the pathogenesis of Six2creFrs2αKO cyst formation. We performed histological assays, Western blots, and quantitative PCR (qPCR). Wh...
متن کاملGα12 is required for renal cystogenesis induced by Pkd1 inactivation.
Mutation of PKD1, encoding the protein polycystin-1 (PC1), is the main cause of autosomal dominant polycystic kidney disease (ADPKD). The signaling pathways downstream of PC1 in ADPKD are still not fully understood. Here, we provide genetic evidence for the necessity of Gα12 (encoded by Gna12, hereafter Gα12) for renal cystogenesis induced by Pkd1 knockout. There was no phenotype in mice with d...
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ژورنال
عنوان ژورنال: Journal of the American Society of Nephrology
سال: 2011
ISSN: 1046-6673,1533-3450
DOI: 10.1681/asn.2011111121