NSMCE2

NSE2 SUMO ligase component of SMC5/6 complex

Summary

This gene encodes a member of a family of E3 small ubiquitin-related modifier (SUMO) ligases that mediates the attachment of a SUMO protein to proteins involved in nuclear transport, transcription, chromosome segregation and DNA repair. The encoded protein is part of the structural maintenance of chromosomes (SMC) 5/6 complex which plays a key role genome maintenance, facilitating chromosome segregation and suppressing mitotic recombination. A knockout of the orthologous mouse gene is lethal prior to embryonic day 10.5. Naturally occurring mutations in this gene, that abolish the SUMO ligase activity, are associated with primordial dwarfism and extreme insulin resistance. [provided by RefSeq, Mar 2017]

Known Variants79 total

rsidPosition (GRCh37)AllelesClassClinVar
rs25374498488:126,114,592C/Gpathogenic
rs7678572448:126,114,606A/Guncertain significance
rs14078641568:126,114,608T/Clikely benign
rs7733860178:126,114,610G/Auncertain significance
rs5426125918:126,114,619C/Tuncertain significance
rs1424112888:126,114,635G/Alikely benign
rs25374503838:126,114,656A/Glikely benign
rs1478754298:126,114,686G/Cuncertain significance
rs3749964198:126,114,690A/Guncertain significance
rs25374510118:126,114,717G/Auncertain significance
rs25376460508:126,163,393G/Alikely benign
rs771131238:126,163,396A/Gbenign
rs25376461508:126,163,399T/Glikely benign
rs2004841328:126,163,425G/Auncertain significance
rs14538870228:126,163,436A/Guncertain significance
rs12469812088:126,163,469G/Auncertain significance
rs7497407368:126,163,471T/Auncertain significance
rs2019037228:126,163,472C/Tlikely benign
rs3777032358:126,163,473G/Auncertain significance
rs25376471818:126,163,495G/Alikely benign
rs12647782998:126,194,331G/Alikely benign
rs7672890958:126,194,334G/Alikely benign
rs7502271798:126,194,336C/Alikely benign
rs7802768078:126,194,344G/Alikely pathogenic
rs7551272158:126,194,357C/Tuncertain significance
rs1996276128:126,194,371A/Guncertain significance
rs1501686498:126,194,457T/Gbenign
rs9527126708:126,194,459G/Tuncertain significance
rs1138993878:126,194,464G/Alikely benign
rs7501701998:126,194,473A/Glikely benign
rs7605146638:126,194,474C/Tpathogenic
rs7789847368:126,194,507A/Glikely benign
rs25377983678:126,194,518T/Alikely benign
rs1462375028:126,194,525C/Tlikely benign
rs70084828:126,267,630T/Gintron variant
rs1881485068:126,316,827G/Aintron variant
rs70157538:126,330,807A/Gintron variant
rs1890742708:126,336,909C/Tintron variant
rs117786838:126,338,189C/G
rs69893838:126,342,923C/Tregulatory region variant
rs70103948:126,343,520T/A
rs69906228:126,343,563G/C
rs28916778:126,344,208C/Tintron variant
rs796344158:126,344,793C/Tintron variant
rs101001218:126,349,130A/Gintron variant
rs3720163168:126,369,443C/Tconflicting classifications of pathogenicity
rs25385263098:126,369,446T/Glikely benign
rs1841675658:126,369,489C/Tlikely benign
rs3743492888:126,369,507T/Guncertain significance
rs768489848:126,369,525C/Tlikely benign
rs25385270878:126,369,539A/Cuncertain significance
rs5631773758:126,369,553A/Guncertain significance
rs11854108498:126,369,565C/Tuncertain significance
rs7708867688:126,369,566C/Tuncertain significance
rs3705849668:126,369,567G/Auncertain significance
rs7743378098:126,369,575C/Tlikely benign
rs3741310708:126,369,577A/Glikely benign
rs25385292348:126,369,936A/Glikely benign
rs21313690768:126,369,988G/Auncertain significance
rs13097728458:126,370,001T/Clikely benign
rs25385296058:126,370,008G/Auncertain significance
rs1153760978:126,370,010C/Tlikely benign
rs7465452428:126,370,011G/Auncertain significance
rs1113598498:126,370,013C/Alikely benign
rs7764697218:126,370,021G/Tuncertain significance
rs2008603518:126,370,022C/Tlikely benign
rs21313693278:126,370,043G/Tuncertain significance
rs1379537408:126,370,044C/Tuncertain significance
rs1424471858:126,370,045G/Auncertain significance
rs13800665038:126,370,056G/Auncertain significance
rs1459985888:126,370,071G/Alikely benign
rs2003933398:126,370,074G/Tlikely benign
rs2019305108:126,379,036C/Tuncertain significance
rs7512671498:126,379,037G/Alikely benign
rs2000749358:126,379,041A/Glikely benign
rs7559587708:126,379,073A/Glikely benign
rs10144466198:126,379,113C/Auncertain significance
rs5367399168:126,379,118T/Clikely benign
rs7771846278:126,379,121C/Tlikely benign

Gene information from NCBI Gene. Variant classifications from ClinVar.