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NSD3

Domain

The KIKL motif recognizes and binds the NET domain of BRD3.

Function

Histone methyltransferase. Preferentially dimethylates 'Lys-4' and 'Lys-27' of histone H3 forming H3K4me2 and H3K27me2. H3 'Lys-4' methylation represents a specific tag for epigenetic transcriptional activation, while 'Lys-27' is a mark for transcriptional repression.

Involvement in disease

Defects in NSD3 may be involved in non small cell lung carcinomas (NSCLC). Amplified or overexpressed in NSCLC.

A chromosomal aberration involving NSD3 is found in childhood acute myeloid leukemia. Translocation t(8;11)(p11.2;p15) with NUP98.

Sequence Similarities

Belongs to the class V-like SAM-binding methyltransferase superfamily. Histone-lysine methyltransferase family. SET2 subfamily.

Tissue Specificity

Highly expressed in brain, heart and skeletal muscle. Expressed at lower level in liver and lung.

Cellular localization

Alternative names

WHSC1L1, DC28, NSD3, Histone-lysine N-methyltransferase NSD3, Nuclear SET domain-containing protein 3, Protein whistle, WHSC1-like 1 isoform 9 with methyltransferase activity to lysine, Wolf-Hirschhorn syndrome candidate 1-like protein 1, WHSC1-like protein 1

swissprot:Q9BZ95 omim:607083 entrezGene:54904