{"database":"GEO","file_versions":[{"headers":{"Content-Type":["application/json"]},"body":{"files":{"Other":["ftp://ftp.ncbi.nlm.nih.gov/geo/series/GSE337nnn/GSE337067/"]},"type":"primary"},"statusCode":"OK","statusCodeValue":200}],"scores":null,"additional":{"omics_type":["Transcriptomics"],"species":["Mus musculus"],"gds_type":["Expression profiling by high throughput sequencing"],"full_dataset_link":["https://www.ncbi.nlm.nih.gov/geo/query/acc.cgi?acc=GSE337067"],"repository":["GEO"],"entry_type":["GSE"],"additional_accession":[]},"is_claimable":false,"name":"Kidney-specific deletion of the BicC family RNA binding protein 1 triggers an ADPKD-like cystogenic program","description":"Autosomal dominant polycystic kidney disease (ADPKD) arises from mutations in polycystin-1/PKD1 or polycystin-2/PKD2 that induce injury-repair pathways and dysregulate cAMP and YAP/TAZ signaling to promote cyst growth. Cystic kidneys with elevated cAMP levels also develop in mouse embryos lacking the BicC family RNA-binding protein 1 (Bicc1). To prevent embryonic lethality, we flanked Bicc1 exon 4 with loxP sites for conditional knockout (cKO) in renal tubules using inducible Pax8rtTA-driven Cre in adults, or distal segment-specific deletion in utero using Ksp-Cre. Whereas Bicc1-deficient adult kidneys formed relatively few cysts within six months, distal nephron-specific deletion induced aggressive PKD-like disease. Automated Renal Tubule Cyst Quantification (ARTCyQ), a novel machine-learning pipeline to analyze immunofluorescent stainings, revealed increasing ambiguity in nephron segment identity and activation of ADPKD-related pathways, including YAP signaling. Accordingly, transcriptional profiling revealed overlap with PKD and injury-repair signatures, supporting a role for Bicc1 in polycystin signaling.","dates":{"publication":"2026/06/30"},"accession":"GSE337067","cross_references":{"GSM":["GSM9847676","GSM9847677","GSM9847674","GSM9847675","GSM9847669","GSM9847678","GSM9847679","GSM9847680","GSM9847672","GSM9847683","GSM9847673","GSM9847684","GSM9847670","GSM9847681","GSM9847682","GSM9847671"],"GPL":["24247"],"GSE":["337067"],"taxon":["Mus musculus"]}}