Ablation of the epithelial-specific splicing factor Esrp1 results in ureteric branching defects and reduced nephron number.

ABSTRACT:

Background

Abnormalities in ureteric bud (UB) branching morphogenesis lead to congenital anomalies of the kidney and reduced nephron numbers associated with chronic kidney disease (CKD) and hypertension. Previous studies showed that the epithelial fibroblast growth factor receptor 2 (Fgfr2) IIIb splice variant supports ureteric morphogenesis in response to ligands from the metanephric mesenchyme during renal organogenesis. The epithelial-specific splicing regulator Esrp1 is required for expression of Fgfr2-IIIb and other epithelial-specific splice variants. Our objective was to determine whether Esrp1 is required for normal kidney development.

Results

Ablation of Esrp1 in mice, alone or together with its paralog Esrp2, was associated with reduced kidney size and increased in

SUBMITTER: Bebee TW 

PROVIDER: S-EPMC5096029 | biostudies-literature | 2016 Oct

REPOSITORIES: biostudies-literature