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histone-lysine N-methyltransferase EHMT1; Euchromatic histone-lysine N-methyltransferase 1; Eu-HMTase1; G9a-like protein 1; GLP; GLP1; histone H3-K9 methyltransferase 5; H3-K9-HMTase 5; lysine N-methyltransferase 1D (EHMT1 EUHMTASE1 GLP KIAA1876 KMT1D)

Function: - histone methyltransferase - specifically mono- & dimethylates Lys-9 of histone H3 (H3K9me1 & H3K9me2, respectively) in euchromatin - H3K9me represents a specific tag for epigenetic transcriptional repression by recruiting HP1 proteins to methylated histones - weakly methylates Lys-27 of histone H3 (H3K27me) - also required for DNA methylation - the histone methyltransferase activity is not required for DNA methylation, suggesting that these 2 activities function independently probably targeted to histone H3 by different DNA-binding proteins like E2F6, MGA, MAX &/or DP1 - during G0 phase, probably contributes to silencing of MYC- & E2F-responsive genes, suggesting a role in G0/G1 transition in cell cycle. - in addition to histone methyltransferase activity, also methylates non-histone proteins: - mediates dimethylation of Lys-373 of p53/TP53 - phosphorylated upon DNA damage, probably by ATM or ATR - heterodimer; heterodimerizes with EHMT2/G9a - interacts with WIZ & EHMT2. part of the E2F6.com-1 complex in G0 phase - interacts (via ANK repeats) with RELA (when monomethylated at Lys-310) S-adenosyl-L-methionine + L-lysine-[histone] = S-adenosyl-L-homocysteine + N(6)-methyl-L-lysine-[histone] Inhibition: - methyltransferase activity is inhibited by BIX-01294 - efficiently inhibited by compound E72, a BIX-01294 derivative Structure: - the ANK repeats recognize & bind RELA subunit of NF-kappa-B, when RELA is monomethylated at Lys-310 (putative) - they also specifically recognize & bind H3K9me1 & H3K9me2 - the SET domain mediates interaction with WIZ - belongs to the histone-lysine methyltransferase family - contains 8 ANK repeats - contains 1 pre-SET domain - contains 1 SET domain Compartment: - nucleus, chromosome - associates with euchromatic regions Alternative splicing: named isoforms=4 Expression: widely expressed Pathology: - defects in EHMT1 are the cause of chromosome 9q subtelomeric deletion syndrome

General

ankyrin repeat domain protein (ANKRD) histone N-methyltransferase phosphoprotein

Properties

SIZE: entity length = 1298 aa MW = 141 kD COMPARTMENT: cell nucleus MOTIF: glutamate-rich region {406-409} MOTIF: glutamate residue (SEVERAL) arginine-rich region {442-449} MOTIF: arginine residue (SEVERAL) Ser phosphorylation site {S497} ankyrin repeat NAME: ankyrin repeat SITE: 737-766 ankyrin repeat NAME: ankyrin repeat SITE: 772-801 ankyrin repeat NAME: ankyrin repeat SITE: 805-834 ankyrin repeat NAME: ankyrin repeat SITE: 838-868 ankyrin repeat NAME: ankyrin repeat SITE: 872-901 ankyrin repeat NAME: ankyrin repeat SITE: 905-934 MOTIF: H3K9me binding {905-907} ankyrin repeat NAME: ankyrin repeat SITE: 938-967 ankyrin repeat NAME: ankyrin repeat SITE: 971-1004 pre-SET domain {1060-1123} MOTIF: Zn+2-binding site SITE: 1062-1062 Zn+2-binding site SITE: 1064-1064 Zn+2-binding site SITE: 1068-1068 Zn+2-binding site SITE: 1073-1073 Zn+2-binding site SITE: 1075-1075 Zn+2-binding site SITE: 1105-1105 Zn+2-binding site SITE: 1109-1109 Zn+2-binding site SITE: 1111-1111 Zn+2-binding site SITE: 1115-1115 SET domain {1125-1247} MOTIF: SAM binding {1136-1138} binding site SITE: 1155-1155 FOR-BINDING-OF: Histone H3K9me H3 interaction {1162-1181} binding site SITE: 1173-1173 FOR-BINDING-OF: S-adenosylmethionine SAM binding {1200-1201} Zn+2-binding site SITE: 1203-1203 H3 interaction {1242-1245} Zn+2-binding site SITE: 1256-1256 binding site SITE: 1257-1257 FOR-BINDING-OF: S-adenosylmethionine Zn+2-binding site SITE: 1258-1258 Zn+2-binding site SITE: 1263-1263 alanine-rich region {1292-1295} MOTIF: alanine residue (SEVERAL)

Database Correlations

OMIM correlations MORBIDMAP 607001 UniProt Q9H9B1 PFAM correlations Entrez Gene 79813 Kegg hsa:79813 ENZYME correlations

References

UniProt :accession Q9H9B1