THE MOUSE NECDIN GENE IS EXPRESSED FROM THE PATERNAL ALLELE ONLY AND LIES IN THE 7C REGION OF THE MOUSE-CHROMOSOME-7, A REGION OF CONSERVEDSYNTENY TO THE HUMAN PRADER-WILLI-SYNDROME REGION
F. Watrin et al., THE MOUSE NECDIN GENE IS EXPRESSED FROM THE PATERNAL ALLELE ONLY AND LIES IN THE 7C REGION OF THE MOUSE-CHROMOSOME-7, A REGION OF CONSERVEDSYNTENY TO THE HUMAN PRADER-WILLI-SYNDROME REGION, European journal of human genetics, 5(5), 1997, pp. 324-332
Prader-Willi syndrome (PWS) is a neurogenetic disorder resulting from
the loss of paternal expression of gene(s) localized in the 15q11-q12
region. A new human gene encoding a putative protein with high homolog
y to the mouse NECDIN protein has recently been characterized and mapp
ed to chromosome 15q11-q12. It is expressed from the paternal allele o
nly, suggesting its potential involvement in PWS. We now report the lo
calization of the mouse Necdin gene in a region of conserved synteny t
o the human PWS region. We demonstrate the paternal specific expressio
n of Necdin in the mouse central nervous system, and show that parenta
l alleles display a differential methylation profile in the coding reg
ion, Finally, fluorescence in situ hybridization analysis reveals an a
synchronous pattern of replication at the Necdin locus. These results
clearly demonstrate imprinting of the mouse Necdin gene. Mouse models
will be powerful tools in the study of human PWS phenotype and imprint
ing mechanisms.