ICE2

Chr 15

interactor of little elongation complex ELL subunit 2

Also known as: BRCC1, NARG2

ICE2 encodes a component of the little elongation complex that regulates small nuclear RNA gene transcription by RNA polymerases II and III, with the protein localizing to Cajal bodies where ribonucleoprotein complexes assemble. Mutations cause neurodevelopmental disorder with growth retardation, seizures, and speech and language delay, inherited in an autosomal recessive pattern. The gene shows very low constraint against loss-of-function variants, consistent with the recessive inheritance pattern observed clinically.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.84
Clinical SummaryICE2
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
0.84LOEUF
pLI 0.000
Z-score 2.44
OE 0.61 (0.450.84)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.91Z-score
OE missense 1.11 (1.041.20)
554 obs / 497.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.61 (0.450.84)
00.351.4
Missense OE1.11 (1.041.20)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 28 / 45.8Missense obs/exp: 554 / 497.2Syn Z: -0.67

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ICE2 · 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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