Allen P B, Morgan G J, Wiedemann L M
Leukaemia Research Fund Centre, Chester Beatty Laboratories, London, UK.
Baillieres Clin Haematol. 1992 Oct;5(4):897-930. doi: 10.1016/s0950-3536(11)80051-x.
Overwhelming evidence indicates a role for the deregulated ABL protein tyrosine kinase in the aetiology of CML and Ph-positive acute leukaemia. These disorders are characterized by the generation of BCR/ABL fusion proteins with elevated tyrosine kinase activity. Although much is known concerning the transforming potential of ABL proteins in various systems, very little is understood of the normal function and mode of regulation of ABL activity. The mechanism of oncogenic activation is therefore also obscure. In spite of this, our understanding of the molecular details of these chromosomal translocations allows the design of therapies directed against their unique, leukaemia-specific proteins and RNA products.
大量证据表明，失调的ABL蛋白酪氨酸激酶在慢性粒细胞白血病（CML）和Ph阳性急性白血病的病因学中发挥作用。这些疾病的特征是产生具有升高酪氨酸激酶活性的BCR/ABL融合蛋白。尽管在各种系统中关于ABL蛋白的转化潜力已有很多了解，但对ABL活性的正常功能和调节模式却知之甚少。因此，致癌激活的机制也不清楚。尽管如此，我们对这些染色体易位分子细节的了解使得能够设计针对其独特的白血病特异性蛋白质和RNA产物的疗法。
