BCR基因

BCR基因（英語：BCR gene），或称断裂点簇集区（英語：breakpoint cluster region），是bcr-abl融合基因的组成部分之一，其与费城染色体的形成机制有关。 一个22号染色体与9号染色体的相互易位作用而产生的费城染色体，它通常在慢性粒细胞性白血病患者体内发现。22号染色体的断裂点就在BCR基因中。这个易位作用产生了一种基于BCR基因和ABL基因进行编码而成的融合蛋白，其基因就位于9号染色体的断裂点上。尽管bcr-abl融合基因已经被广泛研究，但普通的BCR基因机制仍然不明。其蛋白产生一些丝氨酸或苏氨酸蛋白激酶活动，并是一类p21rac酶活化蛋白。 Bcr-Abl肿瘤蛋白齐聚反应域（Bcr-Abl oncoprotein oligomerisation domain）位于BCR的氨基端（N-terminus），其为BCR-ABL融合蛋白的齐聚反应必不可少的基因之一。Bcr-Abl肿瘤蛋白齐聚反应域包含一个短状氨基端α螺旋（alpha-1），一个灵活的转角（Turn）和一个长状的C型α螺旋（alpha-2）。其螺旋体转角使得两个α螺旋能够保持平行定向。这一单体分子的蛋白质结构域，通过一个反平行的卷轴螺旋（coiled coil）形成为一个蛋白质二聚体（protein dimer）。其中两个α螺旋彼此之间发生了结构域对换，其中一个α-1螺旋氨基端进行交换，并挤压另个单体上的α-2螺旋（alpha-2）。两个蛋白质二聚体随后形成一个四聚体（tetramer）。 现今已经发现BCR基因与以下基因发生蛋白-蛋白交互作用（Protein-protein interaction）：PTPN6（英语：PTPN6）、XPB（英语：XPB）、猫肉瘤癌基因、GRB10（英语：GRB10）、CD117（英语：CD117）、Grb2（英语：Grb2）、MLLT4（英语：MLLT4）、Abl基因（英语：ABL (gene)）、HCK（英语：HCK）、SOS1（英语：SOS1）、PIK3CG（英语：PIK3CG）、CRKL（英语：CRKL）和桩蛋白。 Entrez Gene: BCR breakpoint cluster region.  Zhao X, Ghaffari S, Lodish H, Malashkevich VN, Kim PS. 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