RT Journal Article
SR Electronic
T1 Strain-Dependent Modifier Genes Determine Survival in <em>Zfp423</em> Mice
JF G3: Genes|Genomes|Genetics
FD Genetics Society of America
SP 4241
OP 4247
DO 10.1534/g3.120.401720
VO 10
IS 11
A1 Alcaraz, Wendy A.
A1 Liu, Zheng
A1 Valdes, Phoebe
A1 Chen, Edward
A1 Valdovino Gonzalez, Alan G.
A1 Wade, Shelby
A1 Wong, Cinny
A1 Kim, Eunnie
A1 Chen, Hsiang-Hua M.
A1 Ponn, Alison
A1 Concepcion, Dorothy
A1 Hamilton, Bruce A.
YR 2020
UL http://www.g3journal.org/content/10/11/4241.abstract
AB Zfp423 encodes a transcriptional regulatory protein that interacts with canonical signaling and lineage pathways. Mutations in mouse Zfp423 or its human ortholog ZNF423 are associated with a range of developmental abnormalities reminiscent of ciliopathies, including cerebellar vermis hypoplasia and other midline brain defects. Null mice have reduced viability in most strain backgrounds. Here we show complete lethality on a C57BL/6J background, dominant rescue in backcrosses to any of 13 partner strains, with strain-dependent survival frequencies, and evidence for a BALB/c-derived survival modifier locus on chromosome 5. Survival data indicate both perinatal and postnatal periods of lethality. Anatomical data from a hypomorphic gene trap allele observed on both C57BL/6J and BALB/c congenic backgrounds shows an aggregate effect of background on sensitivity to Zfp423 loss rather than a binary effect on viability.