Kopczyńska Ewa, Junik Roman, Tyrakowski Tomasz
Uniwersytet Mikołaja Kopernika w Toruniu, Collegium Medicum w Bydgoszczy.
Pol Merkur Lekarski. 2006 Feb;20(116):210-3.
Mutations of genes coding effectors of signaling pathway RET/PTC-RAS-RAF-MEK-ERK, involved in cell growth and proliferation, are important in papillary thyroid cancer development. To earlier discovered mutations of RAS and RET/PTC genes, BRAF gene mutation has been recently added. Mutation of BRAF gene appears in various types of carcinomas, but most frequently in malignant melanomas (66%) and papillary thyroid cancer (average 44%). The BRAF gene protein product belongs to the serine-threonine kinase family and to the RAF proteins subfamily, among which it is the strongest activator of MAP kinases cascade. The most frequently mutation of BRAF gene is thymine to adenine transversion at nucleotide position 1796 (T1796A). This point mutation causes valine to glutamic acid substitution at residue 599 (V599E), that results in constitutive and oncogenic activation of BRAF kinase. The relation between mutations of BRAF, RAS and RET/PTC genes has not been found, although they together exist in two thirds of papillary thyroid cancers. BRAF(TI796A) oncogene appears in papillary thyroid cancer, whereas it has not been found in follicular thyroid cancer and benign thyroid adenomas. For this reason mutated BRAF gene could be specific molecular marker, with relatively high sensitivity in diagnostics of papillary thyroid cancer. In addition, BRAF gene has been demonstrated as a novel prognostic biomarker, which correlates with unfavorable clinicopathological factors, such as extrathyroidal invasion and distant metastasis.
参与细胞生长和增殖的信号通路RET/PTC-RAS-RAF-MEK-ERK效应器编码基因的突变在甲状腺乳头状癌的发生发展中具有重要意义。除了早期发现的RAS和RET/PTC基因突变外,BRAF基因突变最近也被纳入其中。BRAF基因突变出现在各种类型的癌症中,但最常见于恶性黑色素瘤(66%)和甲状腺乳头状癌(平均44%)。BRAF基因的蛋白质产物属于丝氨酸-苏氨酸激酶家族和RAF蛋白亚家族,是其中MAP激酶级联反应的最强激活剂。BRAF基因最常见的突变是核苷酸位置1796处胸腺嘧啶到腺嘌呤的颠换(T1796A)。这种点突变导致第599位残基缬氨酸被谷氨酸取代(V599E),从而导致BRAF激酶的组成性和致癌性激活。虽然BRAF、RAS和RET/PTC基因的突变共同存在于三分之二的甲状腺乳头状癌中,但尚未发现它们之间的关系。BRAF(T1796A)癌基因出现在甲状腺乳头状癌中,而在甲状腺滤泡癌和甲状腺良性腺瘤中未发现。因此,突变的BRAF基因可能是一种特异性分子标志物,在甲状腺乳头状癌的诊断中具有较高的敏感性。此外,BRAF基因已被证明是一种新的预后生物标志物,与甲状腺外侵犯和远处转移等不良临床病理因素相关。