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CLN3基因与疾病研究进展 被引量:1

Advances in research on CLN3 gene and diseases
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摘要 CLN3基因的编码产物是一种凋亡抑制膜蛋白,在正常组织及细胞中CLN3呈低表达状态;Batten病是由于CLN3发生1kb的基因缺失突变引发的;在多种肿瘤中均发现CLN3基因过表达;反义CLN3能封闭某些肿瘤细胞CLN3基因表达,起抗肿瘤作用。CLN3可能通过调节神经酰胺的合成,也可能通过调节下游基因的表达调节细胞凋亡过程,与肿瘤的发生、发展及治疗有着密切关系。 CLN3 gene product is an antiapoptotie membrane protein, the expressions of CLN3 in normal tissues and cells are at very low level. Juvenile-Batten disease is a oeurndegenerative disease caused by accelerated apoptosis of photoreceptors and neurons resulting from deletion of 1.02 kh in the CLN3 gene.A number of observations showed that CLN3 mRNA and protein are overexpressed in a variety of human cancer cell lines. Blocking of CLN3 expression using an adenovirus-expressing antisense CLN3 inhibited growth and viability of cancer cells. CLN3 may regulate apoptosls through modulating eeramide synthesis or the expression of some down stream genes. More importantly, these results suggested that CLN3 is a novel molecular target for the etiology, progression and theraputies of cancer.
出处 《中国癌症杂志》 CAS CSCD 2005年第6期588-590,共3页 China Oncology
关键词 CLN3 细胞凋亡 肿瘤 CLN3 apoptosis tumor
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参考文献25

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同被引文献6

  • 1肖卫纯,何淑雅,Nanbert Zhong.神经元蜡样质脂褐质沉积病[J].实用神经疾病杂志,2005,8(2):91-92. 被引量:7
  • 2Collins J,Holder G E,Herbert H,et al. Batten disease: features to facilitate early diagnosis[ J]. Br J Ophthalmol,2006,90(9) : 1119-1124.
  • 3Dorota N M,Weina J U , Rocksheng Zhong. N-terminal segments are the functional domains of CLN3-encoded battenin for protein interactions[ J]. Journal of Peking University( Health Sciences),2006,38 (1) :40-42.
  • 4Phillips S N, Benedict J W, Weimer J M ,et al. CLN3 ,the protein associated with batten disease : structure, function and localization [J]. J Neurosci Res,2005,79(5) :573-583.
  • 5Ju W, Wronska A, Moroziewicz D N, et al. Genotype-phenotype analyses of classic neuronal ceroid lipofuscinosis (NCLs) : genetic predictions from clinical and pathological findings[ J]. Journal of Peking University (Health Sciences) ,2006,38 (1) :43-50.
  • 6袁云,陈清棠.神经蜡样质脂褐素沉积病[J].中华儿科杂志,2002,40(10):636-639. 被引量:9

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