RETINOBLASTOMA - CLUES TO HUMAN ONCOGENESIS

RETINOBLASTOMA - CLUES TO HUMAN ONCOGENESIS
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DOI:
10.1126/science.6320372
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发表时间:
1984-01-01
期刊:
影响因子:
56.9
通讯作者:
BENEDICT, WF
BENEDICT, WF
中科院分区:
综合性期刊1区
文献类型:
--
作者:
MURPHREE, AL;BENEDICT, WF

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视网膜母细胞瘤基因可以被认为是一类具有“抑制”或“调节”功能的隐性人类癌症基因的模型。该基因两个等位基因的缺失或失活似乎是视网膜母细胞瘤发生的主要机制。这种机制与假定的人类癌基因直接相反,后者被认为在激活或改变后导致肿瘤发生。在遗传了一个不活跃的视网膜母细胞瘤等位基因的患者中,第二原发癌的发生率很高,这也表明该癌症基因在其他几种原发恶性肿瘤的病因中起着关键作用。最后,观察到特定染色体区域的额外非随机拷贝出现在其中一些肿瘤中,这提供了间接证据,表明表达基因(可能是癌基因)可能与视网膜母细胞瘤的发展有关。
The retinoblastoma gene can be considered a model for a class of recessive human cancer genes that have a "suppressor" or "regulatory" function. The loss or inactivation of both alleles of this gene appears to be a primary mechanism in the development of retinoblastoma. Such a mechanism is in direct contrast to that of putative human oncogenes which are thought to induce tumorigenesis following activation or alteration. The high incidence of second primary tumors among patients who inherit one inactive retinoblastoma allele also suggests that this cancer gene plays a key role in the etiology of several other primary malignancies. Finally, the observation that extra nonrandom copies of specific chromosomal regions occur in some of these tumors provides circumstantial evidence that an "expressor" gene (possibly an oncogene) may be involved in retinoblastoma development.