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    WHSC1 Wolf-Hirschhorn syndrome candidate 1 [ Homo sapiens (human) ]

    Gene ID: 7468, updated on 22-May-2013
    Official Symbol
    WHSC1provided by HGNC
    Official Full Name
    Wolf-Hirschhorn syndrome candidate 1provided by HGNC
    Primary source
    HGNC:12766
    Locus tag
    RP11-262P20.3
    See related
    Ensembl:ENSG00000109685; HPRD:04259; MIM:602952; Vega:OTTHUMG00000121147
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Homo sapiens
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
    Also known as
    WHS; NSD2; TRX5; MMSET; REIIBP
    Summary
    This gene encodes a protein that contains four domains present in other developmental proteins: a PWWP domain, an HMG box, a SET domain, and a PHD-type zinc finger. It is expressed ubiquitously in early development. Wolf-Hirschhorn syndrome (WHS) is a malformation syndrome associated with a hemizygous deletion of the distal short arm of chromosome 4. This gene maps to the 165 kb WHS critical region and has also been involved in the chromosomal translocation t(4;14)(p16.3;q32.3) in multiple myelomas. Alternative splicing of this gene results in multiple transcript variants encoding different isoforms. Some transcript variants are nonsense-mediated mRNA (NMD) decay candidates, hence not represented as reference sequences. [provided by RefSeq, Jul 2008]
    Location :
    4p16.3
    Sequence :
    Chromosome: 4; NC_000004.11 (1873123..1983934)
    See WHSC1 in Epigenomics, MapViewer

    Chromosome 4 - NC_000004.11Genomic Context describing neighboring genes Neighboring gene fibroblast growth factor receptor 3 Neighboring gene leucine zipper-EF-hand containing transmembrane protein 1 Neighboring gene small Cajal body-specific RNA 22 Neighboring gene negative elongation factor complex member A Neighboring gene microRNA 943 Neighboring gene chromosome 4 open reading frame 48

    GeneRIFs: Gene References Into Functions What's a GeneRIF?

    4p partial monosomy syndrome

    Summary from GeneReviews: Wolf-Hirschhorn Syndrome Go to GeneReviews

    Disease Characteristics
    Wolf-Hirschhorn syndrome (WHS) is characterized by typical craniofacial features in infancy consisting of 'Greek warrior helmet appearance' of the nose (the broad bridge of the nose continuing to the forehead), microcephaly, high forehead with prominent glabella, ocular hypertelorism, epicanthus, highly arched eyebrows, short philtrum, downturned mouth, micrognathia, and poorly formed ears with pits/tags. All affected individuals have prenatal-onset growth deficiency followed by postnatal growth retardation and hypotonia with muscle underdevelopment. Developmental delay/intellectual disability of variable degree is present in all. Seizures occur in 50% to 100% of children with WHS. Other findings include skeletal anomalies (60%-70%), congenital heart defects (~50%), hearing loss (mostly conductive) (>40%), urinary tract malformations (25%), and structural brain abnormalities (33%).
    Diagnosis Testing
    The diagnosis of WHS is suggested by the characteristic facial appearance, growth delay, psychomotor retardation, and seizures and is confirmed by detection of a deletion of the Wolf-Hirschhorn syndrome critical region (WHSCR, within chromosome 4p16.3 and between 1.4 and 1.9 Mb from the 4p terminus). Conventional G-banded cytogenetic analysis (routine and high-resolution) detects approximately 50%-60% of the deletions in WHS; fluorescence in situ hybridization (FISH) using a WHSCR probe detects more than 95% of deletions in WHS. The majority (~55%) of individuals have a deletion with no other cytogenetic abnormality (a so-called 'pure deletion'); the remaining have a more complicated cytogenetic finding such as ring 4 chromosome, 4p- mosaicism, or a derivative chromosome 4 resulting from an unbalanced translocation. Chromosomal microarray (CMA) can detect all currently known deletions of the WHSCR and can determine if the deletion is "pure" or part of a more complex imbalance more accurately than either FISH or conventional G-band analysis alone.
    Genetic Counseling
    WHS is caused by deletion of the WHSCR of chromosome 4p16.3 by one of several genetic mechanisms. About 50%-60% of individuals with WHS have a de novo pure deletion of 4p16 and about 40%-45% have an unbalanced translocation with both a deletion of 4p and a partial trisomy of a different chromosome arm. These unbalanced translocations may be de novo or inherited from a parent with a balanced rearrangement. The remaining have other complex rearrangements leading to a 4p16.3 deletion (e.g., ring 4). Risks to family members depend on the mechanism of origin of the deletion. Prenatal testing is possible for families in which one parent is known to be a carrier of a chromosome rearrangement involving 4p16.3.
    References
    Products Interactant Other Gene Complex Source Pubs Description
    BioGRID:113306 BioGRID:117743 AATF    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:106575 ACTB    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:106617 ADAR    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:106848 APP    BioGRID  PubMed Reconstituted Complex 
    BioGRID:113306 BioGRID:106862 AR    BioGRID  PubMed Affinity Capture-Western; Reconstituted Complex; Two-hybrid 
    BioGRID:113306 BioGRID:106886 ARHGAP4    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:106994 ATP5B    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:114497 BAZ1B    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:117036 BRD4    BioGRID  PubMed Affinity Capture-MS 
    BioGRID:113306 BioGRID:116060 CD3EAP    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:107424 CDC5L    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:120743 CDKN2AIP    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115778 CHERP    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:107861 CSTF3    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:107880 CTNNB1    BioGRID  PubMed Affinity Capture-Western 
    BioGRID:113306 BioGRID:108019 DDX1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115776 DDX17    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:114404 DDX18    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:114625 DDX21    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:121353 DDX24    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:122302 DDX31    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:108021 DDX5    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:122480 DDX50    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:122503 DDX54    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:121684 DHX37    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:108025 DHX9    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:108431 EWSR1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:113271 EZR    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:108797 FUS    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:117690 GNL3    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:120214 GPATCH4    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:114738 GTF3C4    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109280 HADHA    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109315 HDAC1    BioGRID  PubMed Affinity Capture-Western; Reconstituted Complex 
    BioGRID:113306 BioGRID:109316 HDAC2    BioGRID  PubMed Affinity Capture-Western; Reconstituted Complex 
    BioGRID:113306 BioGRID:109319 HDLBP    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:126025 HIST2H3C    BioGRID  PubMed Protein-peptide 
    BioGRID:113306 BioGRID:109420 HNRNPA1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:128670 HNRNPA3    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109423 HNRNPAB    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109424 HNRNPC    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109425 HNRNPD    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109428 HNRNPH1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109431 HNRNPK    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109432 HNRNPL    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:110751 HNRNPM    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115530 HNRNPR    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109433 HNRNPU    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:128685 HNRNPUL2    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:119128 HP1BP3    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109528 HSD17B4    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109558 HSP90AB1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109561 HSPD1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109654 IFI16    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109821 ILF2    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:109822 ILF3    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:114353 IQGAP1    BioGRID  PubMed Affinity Capture-MS; Affinity Capture-Western 
    BioGRID:113306 BioGRID:116667 KDM1A    BioGRID  PubMed Affinity Capture-Western 
    BioGRID:113306 BioGRID:114139 KHSRP    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:121775 KIAA1967    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:110186 LMNA    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:110187 LMNB1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:118104 LSM1    BioGRID  PubMed Co-fractionation 
    BioGRID:113306 BioGRID:115014 MDC1    BioGRID  PubMed Affinity Capture-Western; Protein-peptide 
    BioGRID:113306 BioGRID:110368 MECP2    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115494 MPHOSPH10    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115770 MYBBP1A    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:110771 NCL    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:123023 NOL10    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:114654 NOLC1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:110904 NONO    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115783 NOP56    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:119631 NOP58    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:110980 NUMA1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115041 NUP93    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:106652 PARP1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:120490 PBRM1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:119622 PCF11    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:116633 PDCD11    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:116848 PDS5A    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:117040 PES1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:111335 PKP2    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:111370 PLRG1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:117396 POLR1A    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:116140 POP1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:111577 PRKDC    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:118151 PRPF19    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:117298 PRPF6    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:116339 PSIP1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:120558 PSPC1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:111697 PTBP1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115417 RAD50    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:111840 RANGAP1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:125936 RAVER1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:111863 RBBP4    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:113869 RBM10    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115700 RBM14    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:121843 RBM25    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:118134 RBMX    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:112099 RPN1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:114137 RRP1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:116829 RRP12    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:116067 RUVBL2    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115082 SART3    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:120456 SDAD1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115580 SF3A1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:117017 SF3B1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:116188 SF3B2    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:117016 SF3B3    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:112319 SFPQ    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:117439 SIN3A    BioGRID  PubMed Affinity Capture-Western 
    BioGRID:113306 BioGRID:116901 SIN3B    BioGRID  PubMed Affinity Capture-Western 
    BioGRID:113306 BioGRID:119602 SIRT7    BioGRID  PubMed Affinity Capture-MS 
    BioGRID:113306 BioGRID:112481 SMARCA4    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:114045 SMARCA5    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:113871 SMC1A    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:120623 STRBP    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:117312 SUN2    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115755 SYNCRIP    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:117003 TARDBP    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:115853 TBL3    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:112930 TIAM1    BioGRID  PubMed Affinity Capture-MS; Affinity Capture-Western 
    BioGRID:113306 BioGRID:112967 TMPO    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:113003 TOP1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:113008 TOP2B    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:117543 TOR1AIP1    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:113164 UBC    BioGRID  PubMed Affinity Capture-MS 
    BioGRID:113306 BioGRID:115939 USP39    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:121342 UTP3    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:113272 VIM    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:113306 WHSC1    BioGRID  PubMed Affinity Capture-Western 
    BioGRID:113306 BioGRID:113353 XRCC5    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:108822 XRCC6    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:119667 ZFR    BioGRID  PubMed Affinity Capture-RNA 
    BioGRID:113306 BioGRID:1205541 tat    BioGRID  PubMed Reconstituted Complex 

    Markers

    Homology

    Clone Names

    • FLJ23286, KIAA1090, MGC176638

    Gene Ontology Provided by GOA

    Function Evidence Code Pubs
    DNA binding IEA
    Inferred from Electronic Annotation
    more info
     
    chromatin binding IEA
    Inferred from Electronic Annotation
    more info
     
    histone-lysine N-methyltransferase activity IEA
    Inferred from Electronic Annotation
    more info
     
    zinc ion binding IEA
    Inferred from Electronic Annotation
    more info
     
    Process Evidence Code Pubs
    anatomical structure morphogenesis TAS
    Traceable Author Statement
    more info
    PubMed 
    atrial septum primum morphogenesis IEA
    Inferred from Electronic Annotation
    more info
     
    atrial septum secundum morphogenesis IEA
    Inferred from Electronic Annotation
    more info
     
    bone development IEA
    Inferred from Electronic Annotation
    more info
     
    membranous septum morphogenesis IEA
    Inferred from Electronic Annotation
    more info
     
    negative regulation of transcription from RNA polymerase II promoter IEA
    Inferred from Electronic Annotation
    more info
     
    transcription, DNA-dependent IEA
    Inferred from Electronic Annotation
    more info
     
    Component Evidence Code Pubs
    chromosome IEA
    Inferred from Electronic Annotation
    more info
     
    cytoplasm IEA
    Inferred from Electronic Annotation
    more info
     
    nuclear membrane IDA
    Inferred from Direct Assay
    more info
     
    nucleolus IDA
    Inferred from Direct Assay
    more info
     
    nucleus IDA
    Inferred from Direct Assay
    more info
     
    Preferred Names
    histone-lysine N-methyltransferase NSD2
    Names
    histone-lysine N-methyltransferase NSD2
    trithorax/ash1-related protein 5
    nuclear SET domain-containing protein 2
    IL5 promoter REII region-binding protein
    probable histone-lysine N-methyltransferase NSD2
    multiple myeloma SET domain containing protein type III
    NP_001035889.1
    NP_015627.1
    NP_579877.1
    NP_579878.1
    NP_579889.1
    NP_579890.1

    RefSeqs maintained independently of Annotated Genomes

    These reference sequences exist independently of genome builds. Explain

    These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

    Genomic

    1. NG_009269.1 RefSeqGene

      Range
      5001..115812
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_001042424.2NP_001035889.1  histone-lysine N-methyltransferase NSD2 isoform 1

      Status: REVIEWED

      Description
      Transcript Variant: This variant (10) contains a novel 5' non-coding exon compared to transcript variant 1, hence has a different 5' UTR. However, it encodes the same isoform (1) as transcript variants 1, 2 and 3.
      Source sequence(s)
      AF071593, BC032731, BC094825, BM982021, CX866583
      Consensus CDS
      CCDS33940.1
      UniProtKB/Swiss-Prot
      O96028
      Conserved Domains (8) summary
      cd00084
      Location:458507
      Blast Score: 124
      HMG-box; High Mobility Group (HMG)-box is found in a variety of eukaryotic chromosomal proteins and transcription factors. HMGs bind to the minor groove of DNA and have been classified by DNA binding preferences. Two phylogenically distinct groups of Class I ...
      COG2940
      Location:9591203
      Blast Score: 231
      COG2940; Proteins containing SET domain [General function prediction only]
      cd05837
      Location:218337
      Blast Score: 442
      MSH6_like; The PWWP domain is present in MSH6, a mismatch repair protein homologous to bacterial MutS. The PWWP domain of histone-lysine N-methyltransferase, also known as Nuclear SET domain-containing protein 3, is also included. Mutations in MSH6 have been ...
      cd05838
      Location:878972
      Blast Score: 416
      WHSC1_related; The PWWP domain was first identified in the WHSC1 (Wolf-Hirschhorn syndrome candidate 1) protein, a protein implicated in Wolf-Hirschhorn syndrome (WHS). When translocated, WHSC1 plays a role in lymphoid multiple myeloma (MM) disease, also known as ...
      smart00317
      Location:10631186
      Blast Score: 397
      SET; SET (Su(var)3-9, Enhancer-of-zeste, Trithorax) domain
      smart00570
      Location:10121062
      Blast Score: 158
      AWS; associated with SET domains
      pfam00628
      Location:833875
      Blast Score: 124
      PHD; PHD-finger
      cl15348
      Location:12421283
      Blast Score: 84
      RING; RING-finger (Really Interesting New Gene) domain, a specialized type of Zn-finger of 40 to 60 residues that binds two atoms of zinc; defined by the 'cross-brace' motif C-X2-C-X(9-39)-C-X(1-3)- H-X(2-3)-(N/C/H)-X2-C-X(4-48)C-X2-C; probably involved in ...
    2. NM_007331.1NP_015627.1  histone-lysine N-methyltransferase NSD2 isoform 4

      Status: REVIEWED

      Description
      Transcript Variant: This variant (8) contains an additional fragment beyond the 3' end of exon 11, and lacks exons 12-25, as compared to variant 1. This fragment shifts the reading frame and introduces an immediate translation termination. This variant encodes isoform 4, which is much shorter and has a different C-terminus from isoform 1.
      Source sequence(s)
      AF083391
      Consensus CDS
      CCDS46999.1
      UniProtKB/Swiss-Prot
      O96028
      Related
      ENSP00000399251, OTTHUMP00000220792, ENST00000420906, OTTHUMT00000366219
      Conserved Domains (2) summary
      cd00084
      Location:458507
      Blast Score: 111
      HMG-box; High Mobility Group (HMG)-box is found in a variety of eukaryotic chromosomal proteins and transcription factors. HMGs bind to the minor groove of DNA and have been classified by DNA binding preferences. Two phylogenically distinct groups of Class I ...
      cd05837
      Location:218337
      Blast Score: 439
      MSH6_like; The PWWP domain is present in MSH6, a mismatch repair protein homologous to bacterial MutS. The PWWP domain of histone-lysine N-methyltransferase, also known as Nuclear SET domain-containing protein 3, is also included. Mutations in MSH6 have been ...
    3. NM_133330.2NP_579877.1  histone-lysine N-methyltransferase NSD2 isoform 1

      Status: REVIEWED

      Description
      Transcript Variant: This variant (1) lacks exon 12, and contains only 24 exons. It encodes the longest isoform (1).
      Source sequence(s)
      AF083386, AI695058
      Consensus CDS
      CCDS33940.1
      UniProtKB/Swiss-Prot
      O96028
      Related
      ENSP00000372351, OTTHUMP00000220796, ENST00000382895, OTTHUMT00000366269
      Conserved Domains (8) summary
      cd00084
      Location:458507
      Blast Score: 124
      HMG-box; High Mobility Group (HMG)-box is found in a variety of eukaryotic chromosomal proteins and transcription factors. HMGs bind to the minor groove of DNA and have been classified by DNA binding preferences. Two phylogenically distinct groups of Class I ...
      COG2940
      Location:9591203
      Blast Score: 231
      COG2940; Proteins containing SET domain [General function prediction only]
      cd05837
      Location:218337
      Blast Score: 442
      MSH6_like; The PWWP domain is present in MSH6, a mismatch repair protein homologous to bacterial MutS. The PWWP domain of histone-lysine N-methyltransferase, also known as Nuclear SET domain-containing protein 3, is also included. Mutations in MSH6 have been ...
      cd05838
      Location:878972
      Blast Score: 416
      WHSC1_related; The PWWP domain was first identified in the WHSC1 (Wolf-Hirschhorn syndrome candidate 1) protein, a protein implicated in Wolf-Hirschhorn syndrome (WHS). When translocated, WHSC1 plays a role in lymphoid multiple myeloma (MM) disease, also known as ...
      smart00317
      Location:10631186
      Blast Score: 397
      SET; SET (Su(var)3-9, Enhancer-of-zeste, Trithorax) domain
      smart00570
      Location:10121062
      Blast Score: 158
      AWS; associated with SET domains
      pfam00628
      Location:833875
      Blast Score: 124
      PHD; PHD-finger
      cl15348
      Location:12421283
      Blast Score: 84
      RING; RING-finger (Really Interesting New Gene) domain, a specialized type of Zn-finger of 40 to 60 residues that binds two atoms of zinc; defined by the 'cross-brace' motif C-X2-C-X(9-39)-C-X(1-3)- H-X(2-3)-(N/C/H)-X2-C-X(4-48)C-X2-C; probably involved in ...
    4. NM_133331.2NP_579878.1  histone-lysine N-methyltransferase NSD2 isoform 1

      Status: REVIEWED

      Description
      Transcript Variant: This variant (2) lacks exon 2 in the 5' UTR, as compared to variant 1. It encodes the same isoform (1) as variants 1 and 3.
      Source sequence(s)
      AF083387, AI695058
      Consensus CDS
      CCDS33940.1
      UniProtKB/Swiss-Prot
      O96028
      Related
      ENSP00000372348, ENST00000382892
      Conserved Domains (8) summary
      cd00084
      Location:458507
      Blast Score: 124
      HMG-box; High Mobility Group (HMG)-box is found in a variety of eukaryotic chromosomal proteins and transcription factors. HMGs bind to the minor groove of DNA and have been classified by DNA binding preferences. Two phylogenically distinct groups of Class I ...
      COG2940
      Location:9591203
      Blast Score: 231
      COG2940; Proteins containing SET domain [General function prediction only]
      cd05837
      Location:218337
      Blast Score: 442
      MSH6_like; The PWWP domain is present in MSH6, a mismatch repair protein homologous to bacterial MutS. The PWWP domain of histone-lysine N-methyltransferase, also known as Nuclear SET domain-containing protein 3, is also included. Mutations in MSH6 have been ...
      cd05838
      Location:878972
      Blast Score: 416
      WHSC1_related; The PWWP domain was first identified in the WHSC1 (Wolf-Hirschhorn syndrome candidate 1) protein, a protein implicated in Wolf-Hirschhorn syndrome (WHS). When translocated, WHSC1 plays a role in lymphoid multiple myeloma (MM) disease, also known as ...
      smart00317
      Location:10631186
      Blast Score: 397
      SET; SET (Su(var)3-9, Enhancer-of-zeste, Trithorax) domain
      smart00570
      Location:10121062
      Blast Score: 158
      AWS; associated with SET domains
      pfam00628
      Location:833875
      Blast Score: 124
      PHD; PHD-finger
      cl15348
      Location:12421283
      Blast Score: 84
      RING; RING-finger (Really Interesting New Gene) domain, a specialized type of Zn-finger of 40 to 60 residues that binds two atoms of zinc; defined by the 'cross-brace' motif C-X2-C-X(9-39)-C-X(1-3)- H-X(2-3)-(N/C/H)-X2-C-X(4-48)C-X2-C; probably involved in ...
    5. NM_133334.2NP_579889.1  histone-lysine N-methyltransferase NSD2 isoform 3

      Status: REVIEWED

      Description
      Transcript Variant: This variant (7) is missing most of the exons from the 3' end compared to variant 1. It has a different 3' UTR and encodes a shorter isoform (3) with a different C-terminus compared to isoform 1.
      Source sequence(s)
      AF071594, AL132868
      Consensus CDS
      CCDS3356.1
      UniProtKB/Swiss-Prot
      O96028
      Related
      ENSP00000381311, ENST00000398261
      Conserved Domains (2) summary
      cd00084
      Location:458507
      Blast Score: 113
      HMG-box; High Mobility Group (HMG)-box is found in a variety of eukaryotic chromosomal proteins and transcription factors. HMGs bind to the minor groove of DNA and have been classified by DNA binding preferences. Two phylogenically distinct groups of Class I ...
      cd05837
      Location:218337
      Blast Score: 440
      MSH6_like; The PWWP domain is present in MSH6, a mismatch repair protein homologous to bacterial MutS. The PWWP domain of histone-lysine N-methyltransferase, also known as Nuclear SET domain-containing protein 3, is also included. Mutations in MSH6 have been ...
    6. NM_133335.3NP_579890.1  histone-lysine N-methyltransferase NSD2 isoform 1

      Status: REVIEWED

      Description
      Transcript Variant: This variant (3) lacks exons 2 and 3 in the 5' UTR, as compared to variant 1. It encodes the same isoform (1) as variants 1 and 2.
      Source sequence(s)
      AF071593, AF083388, AI339675, AI695058
      Consensus CDS
      CCDS33940.1
      UniProtKB/Swiss-Prot
      O96028
      Related
      ENSP00000372347, ENST00000382891
      Conserved Domains (8) summary
      cd00084
      Location:458507
      Blast Score: 124
      HMG-box; High Mobility Group (HMG)-box is found in a variety of eukaryotic chromosomal proteins and transcription factors. HMGs bind to the minor groove of DNA and have been classified by DNA binding preferences. Two phylogenically distinct groups of Class I ...
      COG2940
      Location:9591203
      Blast Score: 231
      COG2940; Proteins containing SET domain [General function prediction only]
      cd05837
      Location:218337
      Blast Score: 442
      MSH6_like; The PWWP domain is present in MSH6, a mismatch repair protein homologous to bacterial MutS. The PWWP domain of histone-lysine N-methyltransferase, also known as Nuclear SET domain-containing protein 3, is also included. Mutations in MSH6 have been ...
      cd05838
      Location:878972
      Blast Score: 416
      WHSC1_related; The PWWP domain was first identified in the WHSC1 (Wolf-Hirschhorn syndrome candidate 1) protein, a protein implicated in Wolf-Hirschhorn syndrome (WHS). When translocated, WHSC1 plays a role in lymphoid multiple myeloma (MM) disease, also known as ...
      smart00317
      Location:10631186
      Blast Score: 397
      SET; SET (Su(var)3-9, Enhancer-of-zeste, Trithorax) domain
      smart00570
      Location:10121062
      Blast Score: 158
      AWS; associated with SET domains
      pfam00628
      Location:833875
      Blast Score: 124
      PHD; PHD-finger
      cl15348
      Location:12421283
      Blast Score: 84
      RING; RING-finger (Really Interesting New Gene) domain, a specialized type of Zn-finger of 40 to 60 residues that binds two atoms of zinc; defined by the 'cross-brace' motif C-X2-C-X(9-39)-C-X(1-3)- H-X(2-3)-(N/C/H)-X2-C-X(4-48)C-X2-C; probably involved in ...

    RefSeqs of Annotated Genomes: Homo sapiens Annotation Release 104

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference GRCh37.p10 Primary Assembly

    Genomic

    1. NC_000004.11 Reference GRCh37.p10 Primary Assembly

      Range
      1873123..1983934
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    Alternate HuRef

    Genomic

    1. AC_000136.1 Alternate HuRef

      Range
      1835879..1925293
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    Alternate CHM1_1.0

    Genomic

    1. NC_018915.1 Alternate CHM1_1.0

      Range
      1875932..1965280
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    Suppressed Reference Sequence(s)

    The following Reference Sequences have been suppressed. Explain

    1. NM_014919.1: Suppressed sequence

      Description
      NM_014919.1: This RefSeq was permanently suppressed because it is a nonsense-mediated mRNA decay (NMD) candidate.
    2. NM_133332.1: Suppressed sequence

      Description
      NM_133332.1: This RefSeq was permanently suppressed because it is a nonsense-mediated mRNA decay (NMD) candidate.
    3. NM_133333.1: Suppressed sequence

      Description
      NM_133333.1: This RefSeq was permanently suppressed because it is a nonsense-mediated mRNA decay (NMD) candidate.
    4. NM_133336.2: Suppressed sequence

      Description
      NM_133336.2: This RefSeq was permanently suppressed because currently there is insufficient support for the transcript and the protein.

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