Table 1. SMZL signature Gene symbol . C... | American Society of Hematology

Table 1.

SMZL signature

Gene symbol	C	A	M	BCR/NFκB	Median change	Description
MIG^*					4.52	Glucose signal transduction pathway
MS4A2	+				4.18	B-cell: Fc (crystallizable fragment) IgE receptor, β chain; found on the surface of mast cells and basophils
UBD^*			+		3.71	Ubiquitin D, FAT10, B-cell, and dendritic marker
HLA-DMA					3.22	B-cell: major histocompatibility complex, class II, DM α, expressed in B-cell lysosomes, same as RING6
ARHH^*					3.22	Ras oncogene superfamily member, involved in translocation t(3;4) with BCL6
SYK^*	+		+	+	3.19	Positive effector of BCR-stimulated responses; regulation of intracellular calcium ion
PCDH9					3.09	Member of a subfamily of calcium-dependent cell adhesion and recognition proteins of the cadherin superfamily
E2F5			+		2.75	Transcriptional regulator: E2F4/E2F5 complex and p 107 act as transducers of transforming growth factor β (TGF-β) receptor signals upstream of cyclin-dependent kinase (CDK)
TOSO^*	+				2.72	Regulator of Fas-induced apoptosis in T cells
SELL^*	+				2.64	Selectin L (lymphocyte adhesion molecule 1), key for spleen lymphocyte relocalization
TCL 1A^*					2.54	Gene for α-chain of human T-cell receptor, Akt coactivator
AIM2					2.44	Member of interferon-inducible gene family: expressed in spleen, small intestine, and peripheral blood leukocytes
SP140			+		2.37	Lymphocyte-specific nuclear body protein, increased in myeloid precursor cell lines, undetected in nonlymphoid cell lines
PFTK1			+		2.34	Protein kinase activity, may play a role in meiosis
LPXN^*	+		+		2.30	Leupaxin, highly expressed in the spleen
PIR121					2.29	p53-inducible protein
ITM2A					2.29	Integral membrane protein 2A: gene expressed in human CD34⁺ hematopoietic stem/progenitor cells
PTPRC	+		+	+	2.28	CD45, key role in lymphocyte activation, required for proximal signaling events following triggering of the T - and B-cell antigen (Ag) receptors
NSF					2.24	N-ethylmalcimide-sensitive factor required for vesicle-mediated transport
PTPN1	+		+	+	2.23	Protein-tyrosine phosphatase: important regulatory component in signal transduction, neoplastic transformation, and control of mitotic cycle
RASSF2	+				2.22	Ras association domain family 2
CDW52^*					2.20	CAMPATH-1 antigen
HLA-DMB					2.06	B-cell: major histocompatibility complex, class II, DM β
BIRC3^*	+	+		+	2.05	HIAP1 (human inhibitor of apoptosis protein 1), apoptotic suppressor, interacts with tumor necrosis factor (TNF) receptor-associated factor 1 (TRAF1) and TRAF2
TNFRSF5^*	+	+	+	+	2.05	CD40, expressed in B cells, essential role for T-cell-dependent Ig class switching, memory B-cell development, and GC formation
EIF4B			+		2.01	RNA-binding activity and use in the initiation of protein biosynthesis
BTAF1			+		2.01	Regulates transcription in association with TATA binding protein (TBP)
TRAF3^*	+	+		+	2.00	Signal transducer associated with the cytoplasmic domain of tumor necrosis factor type 2 receptor (TNF-R2); also binds to CD40 and lymphotoxin beta receptor (LTB-R)
TRAF5^*	+	+		+	1.99	Interacts with LTB-R and activates NFκB
AMPD3			+		1.96	Purine metabolism
POU2AF1			+		1.93	Transcriptional coactivator, essential for the Ag B-cell response and GC formation, involved in t(3;11) in a form of B-cell leukemia with BCL6
ADD3					1.93	Binds to calmodulin; a t(10;11), yielding a T-cell acute lymphoblastic leukemia and myeloid markers
EGR2			+		1.90	Early growth response 2, zinc finger transcription factor; DNA binding
WBP1					1.85	Protein-protein interaction; similar function to Src homology 3 (SH3) domain
ENPP2	+		+		1.80	Autotaxin (ATX): Purine metabolism; specific target of cell transformation by v-jun
BTK^*	+	+	+	+	1.71	BCR signaling: signal transduction in B cells and other tissues, essential for B-cell activation
PDE4B	+				1.66	Cyclic adenosine monophosphate (cAMP)-specific phosphodiesterase
ICSBP1			+		1.61	Interferon regulatory factor expressed exclusively in cells of the immune system
LTB^*				+	1.54	BCR signaling: lymphotoxin β (TNF superfamily, member 3)

Gene symbol	C	A	M	BCR/NFκB	Median change	Description
MIG^*					4.52	Glucose signal transduction pathway
MS4A2	+				4.18	B-cell: Fc (crystallizable fragment) IgE receptor, β chain; found on the surface of mast cells and basophils
UBD^*			+		3.71	Ubiquitin D, FAT10, B-cell, and dendritic marker
HLA-DMA					3.22	B-cell: major histocompatibility complex, class II, DM α, expressed in B-cell lysosomes, same as RING6
ARHH^*					3.22	Ras oncogene superfamily member, involved in translocation t(3;4) with BCL6
SYK^*	+		+	+	3.19	Positive effector of BCR-stimulated responses; regulation of intracellular calcium ion
PCDH9					3.09	Member of a subfamily of calcium-dependent cell adhesion and recognition proteins of the cadherin superfamily
E2F5			+		2.75	Transcriptional regulator: E2F4/E2F5 complex and p 107 act as transducers of transforming growth factor β (TGF-β) receptor signals upstream of cyclin-dependent kinase (CDK)
TOSO^*	+				2.72	Regulator of Fas-induced apoptosis in T cells
SELL^*	+				2.64	Selectin L (lymphocyte adhesion molecule 1), key for spleen lymphocyte relocalization
TCL 1A^*					2.54	Gene for α-chain of human T-cell receptor, Akt coactivator
AIM2					2.44	Member of interferon-inducible gene family: expressed in spleen, small intestine, and peripheral blood leukocytes
SP140			+		2.37	Lymphocyte-specific nuclear body protein, increased in myeloid precursor cell lines, undetected in nonlymphoid cell lines
PFTK1			+		2.34	Protein kinase activity, may play a role in meiosis
LPXN^*	+		+		2.30	Leupaxin, highly expressed in the spleen
PIR121					2.29	p53-inducible protein
ITM2A					2.29	Integral membrane protein 2A: gene expressed in human CD34⁺ hematopoietic stem/progenitor cells
PTPRC	+		+	+	2.28	CD45, key role in lymphocyte activation, required for proximal signaling events following triggering of the T - and B-cell antigen (Ag) receptors
NSF					2.24	N-ethylmalcimide-sensitive factor required for vesicle-mediated transport
PTPN1	+		+	+	2.23	Protein-tyrosine phosphatase: important regulatory component in signal transduction, neoplastic transformation, and control of mitotic cycle
RASSF2	+				2.22	Ras association domain family 2
CDW52^*					2.20	CAMPATH-1 antigen
HLA-DMB					2.06	B-cell: major histocompatibility complex, class II, DM β
BIRC3^*	+	+		+	2.05	HIAP1 (human inhibitor of apoptosis protein 1), apoptotic suppressor, interacts with tumor necrosis factor (TNF) receptor-associated factor 1 (TRAF1) and TRAF2
TNFRSF5^*	+	+	+	+	2.05	CD40, expressed in B cells, essential role for T-cell-dependent Ig class switching, memory B-cell development, and GC formation
EIF4B			+		2.01	RNA-binding activity and use in the initiation of protein biosynthesis
BTAF1			+		2.01	Regulates transcription in association with TATA binding protein (TBP)
TRAF3^*	+	+		+	2.00	Signal transducer associated with the cytoplasmic domain of tumor necrosis factor type 2 receptor (TNF-R2); also binds to CD40 and lymphotoxin beta receptor (LTB-R)
TRAF5^*	+	+		+	1.99	Interacts with LTB-R and activates NFκB
AMPD3			+		1.96	Purine metabolism
POU2AF1			+		1.93	Transcriptional coactivator, essential for the Ag B-cell response and GC formation, involved in t(3;11) in a form of B-cell leukemia with BCL6
ADD3					1.93	Binds to calmodulin; a t(10;11), yielding a T-cell acute lymphoblastic leukemia and myeloid markers
EGR2			+		1.90	Early growth response 2, zinc finger transcription factor; DNA binding
WBP1					1.85	Protein-protein interaction; similar function to Src homology 3 (SH3) domain
ENPP2	+		+		1.80	Autotaxin (ATX): Purine metabolism; specific target of cell transformation by v-jun
BTK^*	+	+	+	+	1.71	BCR signaling: signal transduction in B cells and other tissues, essential for B-cell activation
PDE4B	+				1.66	Cyclic adenosine monophosphate (cAMP)-specific phosphodiesterase
ICSBP1			+		1.61	Interferon regulatory factor expressed exclusively in cells of the immune system
LTB^*				+	1.54	BCR signaling: lymphotoxin β (TNF superfamily, member 3)

The most highly expressed genes in the SMZL series are shown. The functional signatures cell communication (C), apoptosis (A), metabolism (M), and BCR/NF-κB activation are indicated with “+”.

The most important genes.

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