Ezh2 - enhancer of zeste 2 polycomb repressive complex 2 subunit Gene

Mus musculus

Also known as KMT6; Enx-1; Enx1h; mKIAA4065

Gene ID: 14056 | Gene type: protein coding

About Ezh2

Chromosome 6: 47,507,073-47,572,275 reverse strand.GRCm39:CM000999.3

This gene has 15 transcripts (splice variants), 237 orthologues, 20 paralogues and is associated with 24 phenotypes. Biased expression in CNS E11.5 (RPKM 46.4), CNS E14 (RPKM 3.5) and 11 other tissues.

Summary

Enables several functions, including nucleic acid binding activity; protein methyltransferase activity; and transcription corepressor activity. Involved in B cell differentiation; cell development; and regulation of circadian rhythm. Acts upstream of or within several processes, including macromolecule methylation; negative regulation of cell differentiation; and regulation of transcription, DNA-templated. Located in pericentric heterochromatin and pronucleus. Part of ESC/E(Z) complex and chromatin silencing complex. Is expressed in several structures, including alimentary system; central nervous system; early conceptus; genitourinary system; and integumental system. Used to study B-cell lymphoma; Weaver syndrome; and acute lymphoblastic leukemia. Human ortholog(s) of this gene implicated in Weaver syndrome; bone Cancer (multiple); gastrointestinal system Cancer (multiple); hematologic Cancer (multiple); and lung Cancer. Orthologous to human EZH2 (enhancer of zeste 2 polycomb repressive complex 2 subunit). [provided by Alliance of Genome Resources, Apr 2022]

Ezh2 Products(48)

mRNA	Protein	Name
NM_001409815.1	NP_001396744.1	histone-lysine N-methyltransferase EZH2 isoform 1
NM_001409818.1	NP_001396747.1	histone-lysine N-methyltransferase EZH2 isoform 4
NM_001409813.1	NP_001396742.1	histone-lysine N-methyltransferase EZH2 isoform 1
NM_001146689.1	NP_001140161.1	histone-lysine N-methyltransferase EZH2 isoform 2
NM_001409828.1	NP_001396757.1	histone-lysine N-methyltransferase EZH2 isoform 6
NM_001409819.1	NP_001396748.1	histone-lysine N-methyltransferase EZH2 isoform 4
NM_001409810.1	NP_001396739.1	histone-lysine N-methyltransferase EZH2 isoform 3
NM_001409826.1	NP_001396755.1	histone-lysine N-methyltransferase EZH2 isoform 6
NM_007971.2	NP_031997.2	histone-lysine N-methyltransferase EZH2 isoform 1
NM_001409814.1	NP_001396743.1	histone-lysine N-methyltransferase EZH2 isoform 1
NM_001409823.1	NP_001396752.1	histone-lysine N-methyltransferase EZH2 isoform 6
NM_001409816.1	NP_001396745.1	histone-lysine N-methyltransferase EZH2 isoform 1
NM_001409831.1	NP_001396760.1	histone-lysine N-methyltransferase EZH2 isoform 9
NM_001409817.1	NP_001396746.1	histone-lysine N-methyltransferase EZH2 isoform 1
NM_001409829.1	NP_001396758.1	histone-lysine N-methyltransferase EZH2 isoform 7
NM_001409825.1	NP_001396754.1	histone-lysine N-methyltransferase EZH2 isoform 6
NM_001409822.1	NP_001396751.1	histone-lysine N-methyltransferase EZH2 isoform 5
NM_001409824.1	NP_001396753.1	histone-lysine N-methyltransferase EZH2 isoform 6
NM_001409820.1	NP_001396749.1	histone-lysine N-methyltransferase EZH2 isoform 4
NM_001409827.1	NP_001396756.1	histone-lysine N-methyltransferase EZH2 isoform 6
NM_001409812.1	NP_001396741.1	histone-lysine N-methyltransferase EZH2 isoform 3
NM_001409830.1	NP_001396759.1	histone-lysine N-methyltransferase EZH2 isoform 8
NM_001409811.1	NP_001396740.1	histone-lysine N-methyltransferase EZH2 isoform 3
NM_001409821.1	NP_001396750.1	histone-lysine N-methyltransferase EZH2 isoform 4
NM_001146689.1	NP_001140161.1	histone-lysine N-methyltransferase EZH2 isoform 2
NM_001409810.1	NP_001396739.1	histone-lysine N-methyltransferase EZH2 isoform 3
NM_001409811.1	NP_001396740.1	histone-lysine N-methyltransferase EZH2 isoform 3
NM_001409812.1	NP_001396741.1	histone-lysine N-methyltransferase EZH2 isoform 3
NM_001409813.1	NP_001396742.1	histone-lysine N-methyltransferase EZH2 isoform 1
NM_001409814.1	NP_001396743.1	histone-lysine N-methyltransferase EZH2 isoform 1
NM_001409815.1	NP_001396744.1	histone-lysine N-methyltransferase EZH2 isoform 1
NM_001409816.1	NP_001396745.1	histone-lysine N-methyltransferase EZH2 isoform 1
NM_001409817.1	NP_001396746.1	histone-lysine N-methyltransferase EZH2 isoform 1
NM_001409818.1	NP_001396747.1	histone-lysine N-methyltransferase EZH2 isoform 4
NM_001409819.1	NP_001396748.1	histone-lysine N-methyltransferase EZH2 isoform 4
NM_001409820.1	NP_001396749.1	histone-lysine N-methyltransferase EZH2 isoform 4
NM_001409821.1	NP_001396750.1	histone-lysine N-methyltransferase EZH2 isoform 4
NM_001409822.1	NP_001396751.1	histone-lysine N-methyltransferase EZH2 isoform 5
NM_001409823.1	NP_001396752.1	histone-lysine N-methyltransferase EZH2 isoform 6
NM_001409824.1	NP_001396753.1	histone-lysine N-methyltransferase EZH2 isoform 6
NM_001409825.1	NP_001396754.1	histone-lysine N-methyltransferase EZH2 isoform 6
NM_001409826.1	NP_001396755.1	histone-lysine N-methyltransferase EZH2 isoform 6
NM_001409827.1	NP_001396756.1	histone-lysine N-methyltransferase EZH2 isoform 6
NM_001409828.1	NP_001396757.1	histone-lysine N-methyltransferase EZH2 isoform 6
NM_001409829.1	NP_001396758.1	histone-lysine N-methyltransferase EZH2 isoform 7
NM_001409830.1	NP_001396759.1	histone-lysine N-methyltransferase EZH2 isoform 8
NM_001409831.1	NP_001396760.1	histone-lysine N-methyltransferase EZH2 isoform 9
NM_007971.2	NP_031997.2	histone-lysine N-methyltransferase EZH2 isoform 1

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	Reference	Source
enables DNA binding	IDA IDA: Inferred from direct assay	23942234	MGI
enables RNA binding	IDA IDA: Inferred from direct assay	21936910	MGI
enables RNA binding	IPI IPI: Inferred from physical interaction	21123648	MGI
enables RNA polymerase II cis-regulatory region sequence-specific DNA binding	IDA IDA: Inferred from direct assay	24137001	MGI
enables RNA polymerase II core promoter sequence-specific DNA binding	IDA IDA: Inferred from direct assay	20798045	MGI
enables RNA polymerase II core promoter sequence-specific DNA binding	IMP IMP: Inferred from mutant phenotype	16717091	MGI
enables chromatin DNA binding	IDA IDA: Inferred from direct assay	18483221	MGI
enables chromatin binding	IDA IDA: Inferred from direct assay	15520282	MGI
enables histone H3K27 methyltransferase activity	IMP IMP: Inferred from mutant phenotype	16717091	MGI
enables histone methyltransferase activity	IDA IDA: Inferred from direct assay	12900441	MGI
enables lncRNA binding	IDA IDA: Inferred from direct assay	27618650	MGI
enables primary miRNA binding	IDA IDA: Inferred from direct assay	24137001	MGI
enables protein binding	IPI IPI: Inferred from physical interaction	9584197	MGI
enables protein-lysine N-methyltransferase activity	IDA IDA: Inferred from direct assay	25547114	MGI
enables ribonucleoprotein complex binding	IDA IDA: Inferred from direct assay	21172659	MGI
enables sequence-specific DNA binding	IDA IDA: Inferred from direct assay	20956546	MGI
enables transcription corepressor activity	IDA IDA: Inferred from direct assay	19026781	MGI

Biological Process GO Annotation	Evidence	Reference	Source
involved in B cell differentiation	IMP IMP: Inferred from mutant phenotype	23531880	MGI
acts upstream of or within DNA methylation-dependent heterochromatin formation	IMP IMP: Inferred from mutant phenotype	24105743	MGI
acts upstream of or within G1 to G0 transition	IMP IMP: Inferred from mutant phenotype	19303854	MGI
acts upstream of G1/S transition of mitotic cell cycle	IMP IMP: Inferred from mutant phenotype	19303854	MGI
acts upstream of or within cardiac muscle hypertrophy in response to stress	IDA IDA: Inferred from direct assay	24137001	MGI
acts upstream of or within cellular response to hydrogen peroxide	IDA IDA: Inferred from direct assay	20808772	MGI
acts upstream of or within cellular response to trichostatin A	IMP IMP: Inferred from mutant phenotype	24137001	MGI
acts upstream of or within cerebellar cortex development	IMP IMP: Inferred from mutant phenotype	20798045	MGI
acts upstream of epidermal cell differentiation	IMP IMP: Inferred from mutant phenotype	19303854	MGI
involved in facultative heterochromatin formation	IDA IDA: Inferred from direct assay	20064375	MGI
involved in hemopoiesis	IMP IMP: Inferred from mutant phenotype	23531880	MGI
acts upstream of or within hepatocyte homeostasis	IGI IGI: Inferred from genetic interaction	25477280	MGI
acts upstream of keratinocyte differentiation	IMP IMP: Inferred from mutant phenotype	19303854	MGI
acts upstream of or within liver regeneration	IGI IGI: Inferred from genetic interaction	25477280	MGI
acts upstream of or within negative regulation of DNA-binding transcription factor activity	IGI IGI: Inferred from genetic interaction	25547114	MGI
acts upstream of or within negative regulation of G1/S transition of mitotic cell cycle	IMP IMP: Inferred from mutant phenotype	19303854	MGI
acts upstream of or within negative regulation of epidermal cell differentiation	IMP IMP: Inferred from mutant phenotype	19303854	MGI
acts upstream of or within negative regulation of keratinocyte differentiation	IMP IMP: Inferred from mutant phenotype	19303854	MGI
acts upstream of or within negative regulation of stem cell differentiation	IMP IMP: Inferred from mutant phenotype	19303854	MGI
acts upstream of or within negative regulation of striated muscle cell differentiation	IDA IDA: Inferred from direct assay	15520282	MGI
acts upstream of or within negative regulation of transcription by RNA polymerase II	IDA IDA: Inferred from direct assay	24105743	MGI
acts upstream of or within negative regulation of transcription elongation by RNA polymerase II	IMP IMP: Inferred from mutant phenotype	19303854	MGI
acts upstream of or within protein localization to chromatin	IMP IMP: Inferred from mutant phenotype	18311137	MGI
acts upstream of or within regulation of cell population proliferation	IMP IMP: Inferred from mutant phenotype	19303854	MGI
involved in regulation of circadian rhythm	IMP IMP: Inferred from mutant phenotype	16717091	MGI
acts upstream of or within regulation of gene expression	IMP IMP: Inferred from mutant phenotype	20798045	MGI
acts upstream of or within regulation of gliogenesis	IMP IMP: Inferred from mutant phenotype	20798045	MGI
acts upstream of or within regulation of neurogenesis	IMP IMP: Inferred from mutant phenotype	20798045	MGI
acts upstream of or within regulation of protein phosphorylation	IMP IMP: Inferred from mutant phenotype	19303854	MGI
acts upstream of or within regulation of transcription by RNA polymerase II	IMP IMP: Inferred from mutant phenotype	21498568	MGI
acts upstream of or within response to tetrachloromethane	IGI IGI: Inferred from genetic interaction	25477280	MGI
acts upstream of or within skeletal muscle satellite cell maintenance involved in skeletal muscle regeneration	IMP IMP: Inferred from mutant phenotype	21498568	MGI
acts upstream of stem cell differentiation	IMP IMP: Inferred from mutant phenotype	19303854	MGI

Cellular Component GO Annotation	Evidence	Reference	Source
part of ESC/E(Z) complex	IDA IDA: Inferred from direct assay	19026781	MGI
part of chromatin silencing complex	IDA IDA: Inferred from direct assay	26658965	MGI
is active in nucleus	IDA IDA: Inferred from direct assay	23046516	MGI
located in nucleus	IDA IDA: Inferred from direct assay	9584197	MGI
located in pericentric heterochromatin	IDA IDA: Inferred from direct assay	27892467	MGI
located in pronucleus	IDA IDA: Inferred from direct assay	12900441	MGI

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

Ezh2 Protein Structure

EZH2_WD-Binding

PRC2_HTH_1

preSET_CXC

SET_EZH2

0
200
400
600
746 a.a.

Protein Preferred Names

Protein Names

histone-lysine N-methyltransferase EZH2

enhancer of zeste homolog 2

Ezh2 Protein-protein interaction Information

Type Cross Intra	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Reference
Intra	Ezh2	Q6AXH7	Suz12	Mus musculus	Q80U70	Anti Bait CoIP	23624931
Intra	Ezh2	Q6AXH7	Suz12	Mus musculus	Q80U70	Pull Down	23624931

Cross: Cross-species interaction Intra: Intraspecies interaction

Related Agents

Approval

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-13803	Tazemetostat	Inhibitor	99.93%	Yes

Clinical Phase

Bioactive Molecules (2)

Cat. No.	Product Name	Effect	Purity	Phase
HY-145602	Tulmimetostat	Inhibitor	99.79%	Phase 2
HY-13470	GSK126	Inhibitor	99.98%	Phase 1

Pre-clinical Phase

Bioactive Molecules (51)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-12186	3-Deazaneplanocin A hydrochloride	Inhibitor	99.98%	Unkown
HY-13500	GSK343	Inhibitor	99.88%	Unkown
HY-15646	UNC1999	Inhibitor	99.83%	Unkown
HY-10442	3-Deazaneplanocin A	Inhibitor	99.97%	Unkown
HY-148333	MS177	Inhibitor	98.00%	Unkown
HY-133129	MS1943	Inhibitor	98.92%	Unkown
HY-101512	A-395	Inhibitor	99.12%	Unkown
HY-147525	PROTAC EZH2 Degrader-1	Inhibitor	99.44%	Unkown
HY-16986	EPZ011989	Inhibitor	98.79%	Unkown
HY-N5024	Gambogenic acid	Inhibitor	99.94%	Unkown
HY-100846	JQEZ5	Inhibitor	98.08%	Unkown
HY-15555	EPZ005687	Inhibitor	99.46%	Unkown
HY-141877	MS4322	Degrader	98.49%	Unkown
HY-103682	PF-06726304	Inhibitor	98.37%	Unkown
HY-12856	GSK503	Inhibitor	99.52%	Unkown
HY-15573	EI1	Inhibitor	99.78%	Unkown
HY-147090	Tazemetostat de(methylene morpholine)-O-C3-O-C-COOH	Degrader	99.06%	Unkown
HY-157844	NUCC-0226272	Degrader	99.68%	Unkown
HY-162250	MS8847	Inhibitor	99.78%	Unkown
HY-141877B	MS4322 (isomer)		99.22%	Unkown
HY-162384	EPIC-0628	Inhibitor	98.93%	Unkown
HY-136188	UNC2399	Inhibitor	99.62%	Unkown
HY-A0298	EZH2-IN-2	Inhibitor	99.40%	Unkown
HY-101508	GNA002	Inhibitor	98.02%	Unkown
HY-122936	Tanshindiol C	Inhibitor	98.47%	Unkown
HY-111418	EBI-2511	Inhibitor	99.81%	Unkown
HY-119198	NSC745885	Inhibitor	≥98.0%	Unkown
HY-16986A	EPZ011989 trifluoroacetate	Inhibitor	98.71%	Unkown
HY-146999	YM458	Inhibitor	99.16%	Unkown
HY-144881	(S)-HH2853	Inhibitor	/	Unkown
HY-144330	TDI-6118	Inhibitor	/	Unkown
HY-144882	(R)-HH2853	Inhibitor	/	Unkown
HY-134899	CPI-1328	Inhibitor	/	Unkown
HY-12186G	3-Deazaneplanocin A hydrochloride (GMP)	Inhibitor	/	Unkown
HY-103682A	PF-06726304 acetate	Inhibitor	/	Unkown
HY-161083	PARP/EZH2-IN-2	Inhibitor	/	Unkown
HY-156292	IHMT-EZH2-426	Inhibitor	/	Unkown
HY-131246	DM-01	Inhibitor	98.03%	Unkown
HY-169400	HDACs/EZH2-IN-1	Inhibitor	/	Unkown
HY-143616	EZH2-IN-7	Inhibitor	/	Unkown
HY-149463	EZH2-IN-16	Inhibitor	/	Unkown
HY-163288	EZH2/HSP90-IN-29	Inhibitor	/	Unkown
HY-162601	D-01	Inhibitor	/	Unkown
HY-154854	BBDDL2059	Inhibitor	/	Unkown
HY-169958	EZH2-IN-22	Inhibitor	/	Unkown
HY-132885	PARP/EZH2-IN-1	Inhibitor	/	Unkown
HY-163741	EZH2-IN-19	Inhibitor	/	Unkown
HY-141566	EZH2-IN-5	Inhibitor	/	Unkown
HY-139150	EZH2-IN-4	Inhibitor	/	Unkown
HY-122115	DCE_42	Inhibitor	/	Unkown
HY-116605	GSK926	Inhibitor	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Homo sapiens	Ezh2	NCBI	NCBI:2146

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