p73在神经母细胞瘤中的生物学及临床作用

Biological and clinical role of p73 in neuroblastoma.

作者信息

Romani M, Tonini G P, Banelli B, Allemanni G, Mazzocco K, Scaruffi P, Boni L, Ponzoni M, Pagnan G, Raffaghello L, Ferrini S, Croce M, Casciano I

机构信息

Laboratory of Tumor Genetics, Istituto Nazionale per la Ricerca sul Cancro (IST), Largo Rosanna Benzi 10, 16132 Genova, Italy.

出版信息

Cancer Lett. 2003 Jul 18;197(1-2):111-7. doi: 10.1016/s0304-3835(03)00092-2.

DOI:10.1016/s0304-3835(03)00092-2

PMID:12880969

Abstract

The p73 gene is a p53 homologue localized at 1p36.3, a chromosomal region frequently deleted in neuroblastoma. p73 was originally considered an oncosuppressor gene. However, it was soon realized that its mode of action did not resemble that of a classic anti-oncogene. The recent discovery of N-terminal truncated isoforms, with oncogenic properties, showed that p73 has a 'two in one' structure. Indeed, the full-length variants are strong inducers of apoptosis while the truncated isoforms inhibit the pro-apoptotic activity of p53 and of the full-length p73. This review summarizes some aspects of p73 biology with particular reference to its possible role in neuroblastoma.

摘要

p73基因是一种p53同源物，定位于1p36.3，这是一个在神经母细胞瘤中经常缺失的染色体区域。p73最初被认为是一种肿瘤抑制基因。然而，人们很快意识到它的作用模式与经典的抗癌基因不同。最近发现的具有致癌特性的N端截短异构体表明，p73具有“二合一”结构。事实上，全长变体是凋亡的强诱导剂，而截短异构体则抑制p53和全长p73的促凋亡活性。本综述总结了p73生物学的一些方面，特别提及了它在神经母细胞瘤中可能发挥的作用。