Table 3. Mouse models of NUP98 fusion... | American Society of Hematology

Table 3.

Mouse models of NUP98 fusion oncoproteins

Model type	Fusion	Disease and/or key findings	Co-lesion
HSPC transplant	NUP98-HOXA9	MPD progressing to AML, first proved oncogenicity of NUP98 fusions in vivo⁷²	Meis1 (A)⁷² BCR-ABL1(A)¹²² ETV6 (TEL)-PDGFRB (A)¹²² Kmt2a^−/− (D)^106,120 FLT3-ITD (A)¹²⁰
HSPC transplant	NUP98-HOXD13	MPD progressing to leukemia⁷³	Meis1 (A)⁷³
HSPC transplant	NUP98-TOP1	MPD progressing to leukemia⁷⁴	Meis1⁷⁴
HSPC transplant	NUP98-HOXA10	MPD⁷⁵	Meis1 (A)⁷⁵
HSPC transplant	NUP98-NSD1	AML⁷⁶	FLT3-ITD (A)¹¹⁸
HSPC transplant	NUP98-HHEX	AML³⁹
HSPC transplant	NUP98-PRRX1	MPD⁷⁸	Meis1 (A)⁷⁸
HSPC transplant	NUP98-KDM5A	AML^3,77
HSPC transplant	NUP98-HMGB3	MPD⁷⁹
HSPC transplant	NUP98-CCDC28A	MPD with a few mice developing AML⁸⁰
HSPC transplant	NUP98-IQCG	AML⁸¹
HSPC transplant	NUP98-KAT7	CMML⁵²
GEMM	NUP98-HOXA9	MPD progressing to AML⁸²
GEMM	NUP98-HOXD13	MDS progressing to AML⁸³	FLT3-ITD (A)¹¹⁹ Bcl2 (D)¹¹³ Cdkn2b (p15Ink4b)^−/− (A)¹¹⁵ Bbc3 (Puma)^−/− (D)¹¹⁴ Ep300 (p300)^−/− (A)⁹⁸ Crebbp (Cbp)^−/−⁹⁸
GEMM	NUP98-TOP1	AML after very long latency⁸⁴
GEMM	NUP98-RAP1GS1	AML after very long latency⁸⁴
GEMM	NUP98-PHF23	Various acute leukemias⁸⁵
GEMM	NUP98-KMT2A	MDS progressing to AML⁸⁶
CD34⁺	NUP98-HOXA9	Engraftment and myelomonocytic blasts after 5-7 wk⁸⁷
CD34⁺	NUP98-HOXD13	AML⁸⁸	MN1 (A)⁸⁸
CD34⁺	NUP98-KDM5A	AML⁸⁹
PDX	NUP98-KDM5A	Engraftment with primary and recurrence from same patient in all mice tested⁹⁰
PDX	NUP98-NSD1	Engraftment after 5-6 mo³³
PDX	NUP98-NSD1	Multiple distinct patient samples¹⁶²

Model type	Fusion	Disease and/or key findings	Co-lesion
HSPC transplant	NUP98-HOXA9	MPD progressing to AML, first proved oncogenicity of NUP98 fusions in vivo⁷²	Meis1 (A)⁷² BCR-ABL1(A)¹²² ETV6 (TEL)-PDGFRB (A)¹²² Kmt2a^−/− (D)^106,120 FLT3-ITD (A)¹²⁰
HSPC transplant	NUP98-HOXD13	MPD progressing to leukemia⁷³	Meis1 (A)⁷³
HSPC transplant	NUP98-TOP1	MPD progressing to leukemia⁷⁴	Meis1⁷⁴
HSPC transplant	NUP98-HOXA10	MPD⁷⁵	Meis1 (A)⁷⁵
HSPC transplant	NUP98-NSD1	AML⁷⁶	FLT3-ITD (A)¹¹⁸
HSPC transplant	NUP98-HHEX	AML³⁹
HSPC transplant	NUP98-PRRX1	MPD⁷⁸	Meis1 (A)⁷⁸
HSPC transplant	NUP98-KDM5A	AML^3,77
HSPC transplant	NUP98-HMGB3	MPD⁷⁹
HSPC transplant	NUP98-CCDC28A	MPD with a few mice developing AML⁸⁰
HSPC transplant	NUP98-IQCG	AML⁸¹
HSPC transplant	NUP98-KAT7	CMML⁵²
GEMM	NUP98-HOXA9	MPD progressing to AML⁸²
GEMM	NUP98-HOXD13	MDS progressing to AML⁸³	FLT3-ITD (A)¹¹⁹ Bcl2 (D)¹¹³ Cdkn2b (p15Ink4b)^−/− (A)¹¹⁵ Bbc3 (Puma)^−/− (D)¹¹⁴ Ep300 (p300)^−/− (A)⁹⁸ Crebbp (Cbp)^−/−⁹⁸
GEMM	NUP98-TOP1	AML after very long latency⁸⁴
GEMM	NUP98-RAP1GS1	AML after very long latency⁸⁴
GEMM	NUP98-PHF23	Various acute leukemias⁸⁵
GEMM	NUP98-KMT2A	MDS progressing to AML⁸⁶
CD34⁺	NUP98-HOXA9	Engraftment and myelomonocytic blasts after 5-7 wk⁸⁷
CD34⁺	NUP98-HOXD13	AML⁸⁸	MN1 (A)⁸⁸
CD34⁺	NUP98-KDM5A	AML⁸⁹
PDX	NUP98-KDM5A	Engraftment with primary and recurrence from same patient in all mice tested⁹⁰
PDX	NUP98-NSD1	Engraftment after 5-6 mo³³
PDX	NUP98-NSD1	Multiple distinct patient samples¹⁶²

(A), accelerated disease with co-lesion; (D), delayed disease with co-lesion; MPD, myeloproliferative disease; PDX, patient-derived xenograft. See Table 1 for expansion of other abbreviations.

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