Multiple Organ System Defects and Transcriptional Dysregulation in the Nipbl+/− Mouse, a Model of Cornelia de Lange Syndrome
Figure 5
Nipbl transcript levels are reduced 25–30% in Nipbl+/− mice.
(A) Autoradiograms showing Nipbl and Gapdh probes, and protected fragments of 226 (representing Nipbl exons 11 and parts of exons 10 and 12) and 131 bases (Gapdh), respectively. RNA was prepared from livers of two female littermates (N0 generation; age = 119 days). The minor protected band at ∼185 bp (corresponding to the size of exon 11) most likely arises from the presence of unspliced or alternatively spliced mRNA. (B,C) Quantification of Nipbl/Gapdh ratios, from autoradiograms such as in (A), for adult female liver [(B); age = 73 days], and E17.5 brain (C). Mice in each panel are littermates. Hatched bars = wildtype, filled bars = Nipbl+/−. Error bars = SD for triplicate (B) or quadruplicate (C) measurements.