Description | This entry represents the high mobility group (HMG)-box found in NSD2 and similar sequences from vertebrates.Histone-lysine N-methyltransferase NSD2, also known as Nuclear SET domain-containing protein 2 or wolf-Hirschhorn syndrome candidate 1 protein (WHSC1), is overexpressed frequently in the t(4;14) translocation in 15% to 20% of multiple myeloma [ , , ]. It plays important roles in cancer cell proliferation, survival, and tumour growth, through mediating constitutive NF-kappaB signalling via the cytokine autocrine loop [, ]. It also enhances androgen receptor (AR)-mediated transcription []. The principal chromatin-regulatory activity of NSD2 is dimethylation of histone H3 at lysine 36 (H3K36me2). NSD2 is highly overexpressed in several types of human cancers, including small-cell lung cancers, neuroblastoma, carcinomas of stomach and colon, and bladder cancers, and its overexpression tends to be associated with tumour aggressiveness [, , , , ]. It is frequently deleted in Wolf-Hirschhorn syndrome. NSD2 contains a catalytic suppressor of variegation, enhancer of zeste and trithorax (SET) domain, two proline-tryptophan-tryptophan-prolin motif (PWWP) domains, a high mobility group (HMG) box, five PHD (plant-homeodomain) zinc finger motifs, and an NSD-specific Cys-His rich domain (C5HCH). The SET domain is responsible for histone methyltransferase activity. The PWWP, HMG, and PHD domains mediate chromatin interaction and recognition of histone marks. | Name | Nuclear SET domain-containing protein 2, HMG box |
Short Name | HMG-box_NSD2 | Type | Domain |