Mestre Julia, Chaparro-González Lorea, Granada Isabel, Mallo Mar, Cid Emili, Mancini Estefania, Calvete Oriol, Risueño Ruth M, Starczynowski Daniel T, Solé Francesc
MDS Research Group, Josep Carreras Leukaemia Research Institute, ICO- Hospital Germans Trias i Pujol, Universitat Autònoma de Barcelona, Badalona, 08916, Spain.
Facultat de Biociències, Universitat Autònoma de Barcelona, Barcelona, 08193, Spain.
Mol Cytogenet. 2025 Jun 11;18(1):11. doi: 10.1186/s13039-025-00714-7.
Among the human leukemia cell lines described in the literature, only the MDS-L cell line has been definitively established from a patient during the myelodysplastic syndrome (MDS) phase of the disease. However, the limited studies on its genomic complexity have restricted its applicability as an in vitro model for MDS. Here, we aimed to better characterize the chromosomal and genetic alterations of MDS-L. A comprehensive approach was employed combining conventional G banding, multicolor FISH (M-FISH), SNP arrays with the novel Optical Genome Mapping (OGM) technology. In addition, the mutational landscape was defined using targeted next-generation sequencing (NGS). G-banding revealed two karyotypically distinct cell populations, both exhibiting complex karyotypes. Using G-banding and OGM, we identified previously undescribed structural alterations, including der(1)t(1;7)(q11;q11.2), del(1)(q11), der(4)t(4;5)(p16;q11.2), i(5)(p10), der(6)t(6;15)(p21.3;q15), i(8)(q10), der(9)t(9;10)(q34;p11.21), der(19)t(6;19)(p13;p22) and i(22)(q10). Both OGM and SNP microarray analyses detected multiple copy number variants and regions of homozygosity. Chromosome breakpoints were precisely defined by OGM, allowing the identification of gene disruption events. Moreover, M-FISH technique validated the origins of additional chromosomal material observed in the karyotype, identified cryptic rearrangements, and distinguished the two clonal populations within the cell line. Finally, NGS revealed mutations in CEBPA, NRAS, TET2 and TP53 genes associated with MDS pathology. This multi-technique approach has enabled a precise characterization of the MDS-L cell line's genomic complexity, highlighting the unique contributions of each technique in uncovering various genetic alterations and establishing a valuable resource for mechanistic studies and pre-clinical drug development.
在文献中描述的人类白血病细胞系中,只有MDS-L细胞系是在骨髓增生异常综合征(MDS)疾病阶段从患者体内明确建立的。然而,对其基因组复杂性的有限研究限制了它作为MDS体外模型的适用性。在此,我们旨在更好地表征MDS-L的染色体和基因改变。我们采用了一种综合方法,将传统的G显带、多色荧光原位杂交(M-FISH)、单核苷酸多态性阵列(SNP阵列)与新型光学基因组图谱(OGM)技术相结合。此外,使用靶向二代测序(NGS)确定了突变图谱。G显带揭示了两个核型不同的细胞群体,两者均表现出复杂的核型。通过G显带和OGM,我们鉴定出了先前未描述的结构改变,包括der(1)t(1;7)(q11;q11.2)、del(1)(q11)、der(4)t(4;5)(p16;q11.2)、i(5)(p10)、der(6)t(6;15)(p21.3;q15)、i(8)(q10)、der(9)t(9;10)(q34;p11.21)、der(19)t(6;19)(p13;p22)和i(22)(q10)。OGM和SNP微阵列分析均检测到多个拷贝数变异和纯合性区域。OGM精确确定了染色体断点,从而能够识别基因破坏事件。此外,M-FISH技术验证了核型中观察到的额外染色体物质的来源,识别出隐匿重排,并区分了细胞系内的两个克隆群体。最后,NGS揭示了与MDS病理相关的CEBPA、NRAS、TET2和TP53基因中的突变。这种多技术方法能够精确地表征MDS-L细胞系的基因组复杂性,突出了每种技术在揭示各种基因改变以及为机制研究和临床前药物开发建立宝贵资源方面的独特贡献。