Mantle cell lymphoma (MCL) is incurable using current standard therapeutic regimens. CCMCL1 cell line is a new cell culture model of aggressive mantle cell lymphoma (1). Primary MCL cells of a 58 year old man with progressive MCL and hyperleukocytosis were engrafted in NOD-SCID-gamma mice. Splenocytes obtained from the engrafted mouse were cultured in vitro and yielded the CCMCL1 cell line. CCMCL1 cells possess a blastoid nuclear morphology and proliferate readily in suspension culture. CCMCL1 cells express CD19, CD20, and CD45 and are negative for CD3 and CD25.CCMCL1 has the IGH-CCND1 genetic hallmark of MCL and closely resembles the terminal-phase leukemic MCL cells as evidenced by immunophenotyping, karyotyping, IGHV sequence, and whole exome sequencing (WES) (1). WES provides unbiased, direct evidence for conservation of the MCL genome in CCMCL1 at single-nucleotide resolution. WES detected no mutation in genes of the B-cell receptor or phosphatidylinositol 3-kinase pathway, including BTK and AKT. However, CCMCL1 constitutively expressed phosphorylated AKT and BTK, suggesting CCMCL1 is an appropriate model for studies targeting these two pathways in MCL. Other MCL cell lines have mutations or deletions in either ATM or TP53; with this respect CCMCL1 also offers an opportunity for MCL studies without the interference of ATM or TP53 loss.Reference:1. Zhao X, Chen-Kiang S, Shetty S, Di Liberto M, Bodo J, Durkin L, Eng K, Elemento O, Smith MR, Hsi ED (2015) CCMCL1: a new model of aggressive mantle cell lymphoma. Blood 125(17): 2730-2732.
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