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PERP

[p53 apoptosis effector related to PMP-22] The protein is known also as TP53 apoptosis effector. In databanks this protein is known also as KCP1 [keratinocyte-associated protein 1]. The protein has been described also as THW, a protein that acts as a tumor suppressor.

PERP has been identified by Attardi et al (2000) in a screen for genes that are targets of the tumor suppressor p53 and are involved in cell death by apoptosis. PERP is a tetraspan protein localizing to the plasma membrane and shows sequence similarity to the gas-3 protein (PMP22). PERP is expressed in a p53 dependent manner and at high levels in apoptotic cells compared with cells arrested at the G(1) phase of the cell cycle. The expression of PERP causes cell death in fibroblasts. Ihrie et al (2003) have shown that PERP is a mediator of p53-dependent apoptosis also in neurons and thymocytes.

Ihrie et al (2005) have reported that the murine protein is expressed predominantly in epithelial cells during embryogenesis. Knock-out mice lacking expression of PERP die postnatally with grossly aberrant epithelia due to structural defects in desmosomes. Marques et al (2005) have reported that mice lacking PERP in the skin are resistant to papilloma development in response to chemical carcinogenesis.

Nowak et al (2005) have reported that antisense-mediated loss of PERP function in zebrafish leads to increased apoptosis in PERP-expressing cells of the developing skin and notochord, suggesting that in this contect PERP plays an anti-apoptotic role during normal zebrafish development to regulate tissue-specific cell survival.

Paraoan et al (2006) have reported that the downregulation of PERP expression in primary uveal melanomas is associated with aggressive type.

For other entries pertaining to cell death mechanisms see also the Apoptosis Dictionary section of this encyclopedia.

LAST MODIFIED: March 2005

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