霍奇金淋巴瘤双核 Reed-Sternberg 细胞中编码中体蛋白的基因突变。
Mutations in a gene encoding a midbody protein in binucleated Reed-Sternberg cells of Hodgkin lymphoma.
机构信息
Medical Oncology Program, Institute for Stem Cell & Regenerative Medicine, University of Washington, Seattle, WA, USA.
出版信息
Cell Cycle. 2010 Feb 15;9(4):670-5. doi: 10.4161/cc.9.4.10780. Epub 2010 Mar 2.
Abstract
Classical Hodgkin lymphoma (cHL) is a cancer in which malignant "Reed-Sternberg" cells comprise just a fraction of the bulk of the tumor and are characteristically binucleated. We recently identified a novel gene, KLHDC8B, which appears responsible for some familial cases of cHL. KLHDC8B encodes a midbody kelch protein expressed during cytokinesis. Deficiency of KLHDC8B leads to binucleated cells, implicating its involvement in Reed-Sternberg cell formation. Interestingly, other cancer genes, such as BRCA1 and BRCA2, also encode proteins locating to the midbody during cytokinesis, even though their participation in other pathways has received greater attention. Midbody components may be an overlooked source of tumor suppressor genes.
摘要
经典型霍奇金淋巴瘤（cHL）是一种癌症，其中恶性“Reed-Sternberg”细胞仅占肿瘤大部分的一小部分，并且特征性地具有双核。我们最近发现了一个新基因 KLHDC8B，它似乎与一些家族性 cHL 病例有关。KLHDC8B 编码一种在有丝分裂期间表达的中体 Kelch 蛋白。KLHDC8B 的缺失会导致双核细胞，这表明它参与了 Reed-Sternberg 细胞的形成。有趣的是，其他癌症基因，如 BRCA1 和 BRCA2，也编码在有丝分裂期间定位到中体的蛋白质，尽管它们在其他途径中的参与受到了更多的关注。中体成分可能是肿瘤抑制基因被忽视的来源。
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