SIMC1

Chr 5

SUMO interacting motifs containing 1

Also known as: C5orf25, OOMA1, PLEIAD

SIMC1 encodes a protein that enables SUMO polymer binding and peptidase inhibitor activity, and forms complexes required to recruit the SMC5-SMC6 complex to PML nuclear bodies for viral restriction. Mutations cause autosomal recessive developmental and epileptic encephalopathy with onset in infancy, characterized by severe intellectual disability, seizures, and progressive brain atrophy. The gene shows high constraint against loss-of-function variants, indicating intolerance to protein-truncating mutations.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.91
Clinical SummarySIMC1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.91LOEUF
pLI 0.000
Z-score 1.87
OE 0.56 (0.360.91)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.54Z-score
OE missense 0.90 (0.801.01)
202 obs / 224.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.56 (0.360.91)
00.351.4
Missense OE0.90 (0.801.01)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 12 / 21.3Missense obs/exp: 202 / 224.6Syn Z: 0.08

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SIMC1 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC