Article

The fibrocystin C-terminal domain inhibits Src/STAT3 signal induced cystogenesis of kidney epithelia

2025-08-11

Abstract excerpt

<h4>ABSTRACT</h4> Autosomal recessive polycystic kidney disease (ARPKD) is caused by impaired function of fibrocystin/polyductin (FPC) in collecting duct epithelia resulting in cyst formation. We hypothesized that the membrane-bound C-terminal FPC domain (FPCct) is necessary to suppress cystogensis and facilitate epithelial homeostasis. In ARPKD, cystic kidney epithelia are characterized by a secretory phenotype...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: c8ea1f6f-ab5f-575e-8b27-25a71d5b4c8f
DOI: 10.1101/2025.08.08.669180

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

The fibrocystin C-terminal domain inhibits Src/STAT3 signal induced cystogenesis of kidney epitheliaDOI 10.1101/2025.08.08.669180Read

Molecular and cellular pathophysiology of autosomal recessive polycystic kidney disease (ARPKD).Semantic proximity Read

Fibrocystin/Polyductin releases a C-terminal fragment that translocates into mitochondria and prevents cystogenesisSemantic proximity Read

The carboxy‐terminus of the human ARPKD protein fibrocystin can control STAT3 signalling by regulating SRC‐activationSemantic proximity Read

Therapeutic Potential for CFTR Correctors in Autosomal Recessive Polycystic Kidney Disease.Semantic proximity Read

Therapeutic targets for polycystic kidney disease.Semantic proximity Read

JAK2 is highly expressed by cyst-lining cells and regulates cystogenesisSemantic proximity Read

Cystin is required for maintaining fibrocystin (FPC) levels and safeguarding proteome integrity in mouse renal epithelial cells A mechanistic connection between the kidney defects in <i>cpk</i> mice and human ARPKDSemantic proximity Read

Polycystin-2 expression is regulated by a PC2-binding domain in the intracellular portion of fibrocystin.Semantic proximity Read

Fibrocystin/polyductin modulates renal tubular formation by regulating polycystin-2 expression and function.Semantic proximity Read

Tubular STAT3 limits renal inflammation in autosomal dominant polycystic kidney diseaseSemantic proximity Read

c-Jun N-terminal kinase (JNK) signaling contributes to cystic burden in polycystic kidney diseaseSemantic proximity Read

Regulation of STATs by polycystin-1 and their role in polycystic kidney diseaseSemantic proximity Read