Division of Hematology/Oncology, Department of Medicine, University of California, Irvine, Irvine, CA, USA.
Department of Biological Chemistry, University of California, Irvine, Irvine, CA, USA.
Nat Commun. 2024 Jul 27;15(1):6331. doi: 10.1038/s41467-024-50537-0.
Activation-induced cytidine deaminase (AID) is a B cell-specific mutator required for antibody diversification. However, it is also implicated in the etiology of several B cell malignancies. Evaluating the AID-induced mutation load in patients at-risk for certain blood cancers is critical in assessing disease severity and treatment options. We have developed a digital PCR (dPCR) assay that allows us to quantify mutations resulting from AID modification or DNA double-strand break (DSB) formation and repair at sites known to be prone to DSBs. Implementation of this assay shows that increased AID levels in immature B cells increase genome instability at loci linked to chromosomal translocation formation. This includes the CRLF2 locus that is often involved in translocations associated with a subtype of acute lymphoblastic leukemia (ALL) that disproportionately affects Hispanics, particularly those with Latin American ancestry. Using dPCR, we characterize the CRLF2 locus in B cell-derived genomic DNA from both Hispanic ALL patients and healthy Hispanic donors and found increased mutations in both, suggesting that vulnerability to DNA damage at CRLF2 may be driving this health disparity. Our ability to detect and quantify these mutations will potentiate future risk identification, early detection of cancers, and reduction of associated cancer health disparities.
激活诱导胞嘧啶脱氨酶(AID)是一种 B 细胞特异性突变体,是抗体多样化所必需的。然而,它也与几种 B 细胞恶性肿瘤的病因有关。评估有某些血液癌风险的患者的 AID 诱导的突变负荷对于评估疾病严重程度和治疗选择至关重要。我们开发了一种数字 PCR(dPCR)检测方法,该方法允许我们定量分析 AID 修饰或 DNA 双链断裂(DSB)形成和修复导致的突变,这些突变发生在已知易发生 DSB 的部位。该检测方法的实施表明,不成熟 B 细胞中 AID 水平的增加会增加与染色体易位形成相关的基因组不稳定性,这些易位与一种亚型的急性淋巴细胞白血病(ALL)有关,该疾病不成比例地影响西班牙裔人,尤其是具有拉丁美洲血统的西班牙裔人。使用 dPCR,我们对西班牙裔 ALL 患者和健康西班牙裔供体的 B 细胞衍生基因组 DNA 中的 CRLF2 基因座进行了特征分析,发现两者的突变都增加了,这表明 CRLF2 处的 DNA 损伤易感性可能是导致这种健康差异的原因。我们检测和定量这些突变的能力将增强未来的风险识别、癌症的早期检测以及相关癌症健康差异的减少。