Early developmental arrest and impaired gastrointestinal homeostasis in U12-dependent splicing-defective Rnpc3-deficient mice

Systemic Rnpc3 deficiency causes severe atrophy of the entire gastrointestinal epithelium. Histological analysis of the epithelial lining of the esophagus, stomach, small intestine, and colon in tamoxifen-treated control (Rnpc3^lox/lox) (A–D) and experimental (UBC-CreERT2;Rnpc3^lox/lox) mice (E–H) 8 d post-tamoxifen treatment. Whereas the organization and structural integrity of the gastrointestinal epithelium is intact in control organs (A–D), recombination of both Rnpc3^lox alleles causes large-scale degeneration of the epithelial layer interspersed with discrete pockets of regenerating crypt-like structures (arrowheads in F, G, and H). Scale bar in A = 50 µm and applies to all images.