The detailed entries of the Imprinted Gene Catalogue database were created by Benjamin Tycko, Columbia University, New York and are updated by Ian Morison


 

Detailed entry for Gene:

Peg3
Pw1

Chromosome:

Human Chr19q13.4
Mouse Chr7 (proximal)

Links:

OMIM

Imprinting:

The maternal allele of Peg3/Pw1 is silenced; paternal allele active in multiple fetal tissues, and in adult brain and ovary of mice, as well as in several fetal and adult human tissues (Li LL et al, 2000; Murphy SK et al, 2001; Hiby SE et al, 2001; Kuroiwa Y et al, 1996).

Gene Product:

Peg3 encodes a large Kruppel-type zinc finger transcription factor with a proline-rich domain, which is expressed widely during fetal development of mice, and strongly in adult neurons and skeletal muscle (Kuroiwa Y et al, 1996; Relaix F et al, 1998). Human PEG3 has strongest expression in the placenta and ovary, but PEG3 mRNA is also detected in various other tissues (Kim J et al, 1997).

Functional Data:

In cultured cell lines, Peg3/Pw1 associated specifically with TRAF2, a downstream effector of tumor necrosis factor (TNF) signaling, but not with other TRAF family members (Relaix F et al, 1998). Peg3 expression activated NFkappaB via IkappaB-NFkappaB dissociation, and acted synergistically with TRAF2. Peg3/Pw1 is induced during p53-mediated cell death (Deng Y et al, 2000). PEG3 has been proposed as an imprinted tumour suppressor gene, based on its epigenetic silencing and growth-suppressing activity in gliomas (Kohda T et al, 2001; Maegawa S et al, 2001). Data from Peg3-knockout mice indicate that this gene regulates both offspring growth and maternal behaviour (Li L et al, 1999). The Peg3-null offspring were about 20% smaller at birth, and there was markedly reduced nursing behaviour and a reduced number of oxytocin-positive neurons in the hypothalamus of Peg3-null females. The TNF pathway appeared normal in fibroblasts from these knockout mice (Ledgerwood EC et al, 2000).

 

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