ITM2C

Chr 2

integral membrane protein 2C

Also known as: BRI3, BRICD2C, E25, E25C, ITM3

The ITM2C protein negatively regulates amyloid-beta peptide production by inhibiting APP processing through alpha- and beta-secretase pathways. Mutations cause autosomal recessive neurodevelopmental disorders with intellectual disability and developmental delay. The gene shows moderate constraint against loss-of-function variants (pLI 0.70, LOEUF 0.50), suggesting intolerance to complete protein loss.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
2
Pubs (1 yr)
29
P/LP submissions
0%
P/LP missense
0.50
LOEUF
Mechanism
Clinical SummaryITM2C
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.70) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
29 unique Pathogenic / Likely Pathogenic· 41 VUS of 93 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.50LOEUF
pLI 0.704
Z-score 2.78
OE 0.16 (0.060.50)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.38Z-score
OE missense 0.92 (0.811.05)
159 obs / 173.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.16 (0.060.50)
00.351.4
Missense OE0.92 (0.811.05)
00.61.4
Synonymous OE1.04
01.21.6
LoF obs/exp: 2 / 12.7Missense obs/exp: 159 / 173.1Syn Z: -0.28

ClinVar Variant Classifications

93 submitted variants in ClinVar

Classification Summary

Pathogenic29
VUS41
Likely Benign2
Benign2
29
Pathogenic
41
VUS
2
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
29
0
29
Likely Pathogenic
0
0
0
0
0
VUS
0
39
2
0
41
Likely Benign
0
2
0
0
2
Benign
0
0
0
2
2
Total04131274

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

ITM2C · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗