NSMCE2

Chr 8

NSE2 SUMO ligase component of SMC5/6 complex

Also known as: C8orf36, MMS21, NSE2, ZMIZ7

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]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtSeckel syndrome 10

Clinical highlights

Gene-disease validity (ClinGen)
Seckel syndrome 10 · ARModerateconsider for supplementary testing
0
Active trials
8
Pubs (1 yr)
P/LP submissions
P/LP missense
0.86
LOEUF
Mechanism
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.86LOEUF
pLI 0.038
Z-score 1.88
OE 0.38 (0.180.86)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.82Z-score
OE missense 0.80 (0.690.94)
109 obs / 135.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.38 (0.180.86)
00.351.4
Missense OE?0.80 (0.690.94)
00.61.4
Synonymous OE?1.07
01.21.6
LoF obs/exp: 4 / 10.6Missense obs/exp: 109 / 135.7Syn Z: -0.36

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NSMCE2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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