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'Dub'bing a tumor suppressor pathway.

机译:“复制”肿瘤抑制途径。

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摘要

The autosomal recessive disease Fanconi anemia (FA) causes bone marrow failure and a hugely increased propensity to develop cancer. Cells from FA patients are prone to chromosome breakage, indicating that FA gene products are required to ensure genomic integrity. Most of the identified FA proteins are components of a nuclear complex whose principal function is to activate FANCD2 by monoubiquitination. Monoubiquitinated FANCD2 accumulates at sites of genome damage, where it probably functions to facilitate DNA repair. A recent paper in Molecular Cell (Nijmanet al., 2005) reports the identification of an enzyme that is responsible for regulating the FA pathway by deactivating FANCD2.
机译:常染色体隐性遗传疾病Fanconi贫血(FA)会导致骨髓衰竭,并大大增加患癌的可能性。 FA患者的细胞容易发生染色体断裂,表明需要FA基因产物来确保基因组完整性。大多数已鉴定的FA蛋白是核复合物的组成部分,其主要功能是通过单泛素化激活FANCD2。单泛素化的FANCD2聚集在基因组损伤的部位,在该部位可能起到促进DNA修复的作用。分子细胞(Nijmanet等人,2005)上的最新论文报道了一种通过灭活FANCD2来调节FA途径的酶的鉴定。

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