. Author manuscript; available in PMC: 2021 May 4.

Published in final edited form as: Leukemia. 2020 Nov 4;35(2):360–376. doi: 10.1038/s41375-020-01066-4

Table 3.

RBPs involved in mRNA splicing

Gene	Protein/RNP function	Target Genes	Biological consequences	Type of cancer	Expression in cancer	Ref
*SF3B1* *U2AF1(35)* *SRSF2* *ZRSR2* *SF3A1* *PRPF40B*	splicing factors 3’ splice site recognition in pre-mRNA	17 genes NMD (SMG1,5,6,7,8 9, DHX34, UPFs, BTZ, Y14, PYM, hNAG, MAGOH, eIF4A3).	apoptosis, G1/M phase arrest, compromise reconstitution capacity	MDS, HeLa	loss or change-of-function due to mutations and mis-splicing	⁴⁴
*U1 snRNP*	splicing factor 5’ splice site recognition thru U1 snRNA:pre-mRNA base pairing	MSI2, POLD1, CD44, ABCD3, global splicing	mis-splicing, intron retention;downregulated genes related to apoptosis, more aggressive CLL	CLL, HCC	mutations in canonical U1 snRNA genes, change-of-function	⁵⁸
*hnRNPA1*	splicing factor	DICER, NT5C2, global splicing, RNA metabolism	global mis-splicing, BM failure	pediatric B-ALL	loss or change-of-function due to mis-splicing	⁴⁹
*RBM15*	RNA splicing, erythro-megakaryocytic lineage factors	GATA1, RUNX1, MPL, TAL1 RBM15-MKL1	altered splicing, abolished megakaryocytic differentiation	AMKL	deletions, fusion (tumor suppressor?)	²⁷
*RBM39*	splicing factor	HOXA9 transcriptional targets	RBM39 inactivation leads to mis-splicing and downregulation of GATA2, BMI-1, MYB	AML, MOLM-13 (MLL-AF9, FLT3ITD), K562	non-oncogenic “addiction”, upregulated	⁵⁰
*DCPS*	decapping	spliceosomes, transcription, export, nuclear pore complexes	DCPS inactivation causes pre-mRNA mis-splicing, induces a type I interferon response in AML	CALM/AF10 or MLL/AF9 leukemia, AML MOLM-13, AML PDX	AML dependency, upregulated	⁵¹

Abbreviations used: Acute myeloid leukemia (AML); acute promyeloblastic leukemia (APL); acute megakaryoblastic leukemia (AMKL); acute lymphoblastic or lymphocytic (ALL); B-cell acute lymphoblastic leukemia (B-ALL); adult T-cell leukemia/lymphoma (ATL); diffuse large B-cell lymphomas (DLBCLs); chronic myeloid leukemia (CML), chronic phase (CP), accelerated phase (AP), blast crisis (BC); chronic lymphoblastic or lymphocytic leukemia (CLL); myelodysplastic syndromes (MDS); multiple myeloma (MM); hepatocellular carcinoma (HCC); leukemia stem cell (LSC); wild type (WT); patient-derived xenograft (PDX); bone marrow (BM); hematopoietic stem/progenitor cells (HSCs, HSPCs).