Hofstra R M, Cheng N C, Hansen C, Stulp R P, Stelwagen T, Clausen N, Tommerup N, Caron H, Westerveld A, Versteeg R, Buys C H
University of Groningen, Department of Medical Genetics, The Netherlands.
Hum Genet. 1996 Mar;97(3):362-4. doi: 10.1007/BF02185773.
Neuroblastoma occasionally occurs in diseases associated with abnormal neurocrest differentiation, e.g. Hirschsprung disease. Expression studies in developing mice suggest that the proto-oncogene RET plays a role in neurocrest differentiation. In humans expression of RET is limited to certain tumor types, including neuroblastoma, that derive from migrating neural crest cells. Mutations of RET are found associated with Hirschsprung disease. These data prompted us to investigate expression of RET and to search for gene mutations in neuroblastoma. Out of 16 neuroblastoma cell lines analyzed, 9 show clear expression of RET in a Northern blot analysis. In a single strandt conformation polymorphism (SSCP) analysis of all exons, no mutations were detected other than neutral polymorphisms. In a patient with neuroblastoma, from a family in which different neurocrestopathies, including neuroblastoma and Hirschsprung disease, had occurred, we also failed to detect RET mutations. Possibly, expression of RET in neuroblastoma merely reflects the differentiation status of the tumor cells. The absence of mutations suggests that RET does not play a crucial role in the tumorigenesis of neuroblastoma.
神经母细胞瘤偶尔会发生于与神经嵴分化异常相关的疾病中,例如先天性巨结肠症。对发育中小鼠的表达研究表明,原癌基因RET在神经嵴分化中发挥作用。在人类中,RET的表达仅限于某些源自迁移神经嵴细胞的肿瘤类型,包括神经母细胞瘤。已发现RET突变与先天性巨结肠症有关。这些数据促使我们研究RET的表达,并在神经母细胞瘤中寻找基因突变。在分析的16个神经母细胞瘤细胞系中,9个在Northern印迹分析中显示出RET的清晰表达。在对所有外显子的单链构象多态性(SSCP)分析中,除中性多态性外未检测到其他突变。在一个患有神经母细胞瘤的患者中,其家族中曾发生过包括神经母细胞瘤和先天性巨结肠症在内的不同神经嵴病,我们也未能检测到RET突变。可能,神经母细胞瘤中RET的表达仅仅反映了肿瘤细胞的分化状态。未发现突变表明RET在神经母细胞瘤的肿瘤发生中不发挥关键作用。