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首页> 外文期刊>Leukemia and lymphoma >Development and characterization of a novel human Waldenstr?m macroglobulinemia cell line: RPCI-WM1, Roswell Park Cancer Institute- Waldenstr?m Macroglobulinemia 1
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Development and characterization of a novel human Waldenstr?m macroglobulinemia cell line: RPCI-WM1, Roswell Park Cancer Institute- Waldenstr?m Macroglobulinemia 1

机译:新型人类华氏巨球蛋白血症细胞系的开发和鉴定:RPCI-WM1,罗斯威尔公园癌症研究所-华氏巨球蛋白血症1

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摘要

Understanding the biology of Waldenstr?m macroglobulinemia is hindered by a lack of preclinical models. We report a novel cell line, RPCI-WM1, from a patient treated for WM. The cell line secretes human immunoglobulin M (h-IgM) with κ-light chain restriction identical to the primary tumor. The cell line has a modal chromosomal number of 46 and harbors chromosomal changes such as deletion of 6q21, monoallelic deletion of 9p21 (CDKN2A), 13q14 (RB1) and 18q21 (BCL-2), with a consistent amplification of 14q32 (immunoglobulin heavy chain; IgH) identical to its founding tumor sample. The clonal relationship is confirmed by identical CDR3 length and single nucleotide polymorphisms as well as a matching IgH sequence of the cell line and founding tumor. Both also harbor a heterozygous, non-synonymous mutation at amino acid 265 in the MYD88 gene (L265P). The cell line expresses most of the cell surface markers present on the parent cells. Overall, RPCI-WM1 represents a valuable model to study Waldenstr?m macroglobulinemia.
机译:缺乏临床前模型阻碍了对Waldenstr?m巨球蛋白血症生物学的了解。我们从一名接受WM治疗的患者中报告了一种新型细胞系RPCI-WM1。该细胞系分泌具有与原发肿瘤相同的κ轻链限制的人免疫球蛋白M(h-IgM)。该细胞系的模态染色体数为46,并且具有染色体变化,例如缺失6q21,单等位基因缺失9p21(CDKN2A),13q14(RB1)和18q21(BCL-2),并持续扩增14q32(免疫球蛋白重链) ; IgH)与其建立的肿瘤样本相同。克隆关系通过相同的CDR3长度和单核苷酸多态性以及细胞系和发现肿瘤的匹配IgH序列确认。两者都在MYD88基因(L265P)的265位氨基酸处具有杂合的,非同义的突变。细胞系表达存在于亲本细胞上的大多数细胞表面标记。总体而言,RPCI-WM1代表了研究Waldenstr?m巨球蛋白血症的有价值的模型。

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