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Details on Person UniProt:O14686-1 KMT2D

Class:IdReferenceIsoform:401245
_chainChangeLogchain:1-5537 added on Fri February 6 2015
_displayNameUniProt:O14686-1 KMT2D
_timestamp2025-02-21 19:39:23
chainchain:1-5537
checksum31C6DAB0A754F72A
commentFUNCTION Histone methyltransferase that catalyzes methyl group transfer from S-adenosyl-L-methionine to the epsilon-amino group of 'Lys-4' of histone H3 (H3K4) (PubMed:25561738). Part of chromatin remodeling machinery predominantly forms H3K4me1 methylation marks at active chromatin sites where transcription and DNA repair take place (PubMed:17500065, PubMed:25561738). Acts as a coactivator for estrogen receptor by being recruited by ESR1, thereby activating transcription (PubMed:16603732).CATALYTIC ACTIVITY L-lysyl(4)-[histone H3] + S-adenosyl-L-methionine = N(6)-methyl-L-lysyl(4)-[histone H3] + S-adenosyl-L-homocysteine + H(+)SUBUNIT Component of the MLL2 complex (also named ASCOM complex), at least composed of catalytic subunit KMT2D/MLL2, ASH2L, RBBP5, WDR5, NCOA6, DPY30, KDM6A, PAXIP1/PTIP, PAGR1 and alpha- and beta-tubulin (PubMed:12482968, PubMed:16603732, PubMed:17021013, PubMed:17500065, PubMed:17761849, PubMed:17851529, PubMed:23508102). Forms a core complex with the evolutionary conserved subcomplex WRAD composed of WDR5, RBBP5, ASH2L/ASH2 and DPY30 subunits; WRAD differentially stimulates the methyltransferase activity (PubMed:25561738). Interacts with ESR1; interaction is direct (PubMed:16603732). Interacts (via WIN motif) with WDR5 (PubMed:22266653, PubMed:22665483).INTERACTION Expressed in most adult tissues, including a variety of hematoipoietic cells, with the exception of the liver.DOMAIN LXXLL motifs 5 and 6 are essential for the association with ESR1 nuclear receptor.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.MISCELLANEOUS This gene mapped to a chromosomal region involved in duplications and translocations associated with cancer.SIMILARITY Belongs to the class V-like SAM-binding methyltransferase superfamily. Histone-lysine methyltransferase family. TRX/MLL subfamily.CAUTION Another protein KMT2B/MLL4, located on chromosome 19, was first named MLL2 (see AC Q9UMN6). Thus, KMT2B/MLL4 is often referred to as MLL2 and vice versa in the literature.
created[InstanceEdit:400710] Schmidt, EE, 2009-03-25 05:33:35
descriptionrecommendedName: Histone-lysine N-methyltransferase 2D shortName: Lysine N-methyltransferase 2D ecNumber evidence="30"2.1.1.364 alternativeName: ALL1-related protein alternativeName: Myeloid/lymphoid or mixed-lineage leukemia protein 2
geneNameKMT2D
ALR
MLL2
MLL4
identifierO14686
isoformParent
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Alternative splicing
Chromatin regulator
Coiled coil
Congenital hypothyroidism
Deafness
Disease variant
Intellectual disability
Isopeptide bond
Metal-binding
Methylation
Methyltransferase
Nucleus
Phosphoprotein
Proteomics identification
Reference proteome
Repeat
S-adenosyl-L-methionine
Transcription
Transcription regulation
Transferase
Ubl conjugation
Zinc
Zinc-finger
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9841277] Weiser, Joel
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
nameKMT2D
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:9003608] ENSEMBL:ENSG00000167548 KMT2D [Homo sapiens]
secondaryIdentifierKMT2D_HUMAN
O14687
sequenceLength5537
species[Species:48887] Homo sapiens
variantIdentifierO14686-1
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