CRBN

Chr 3AR

cereblon

Also known as: MRT2, MRT2A

CRBN encodes cereblon, a substrate recognition component of a DCX E3 ubiquitin ligase complex that mediates proteasomal degradation of regulatory proteins and maintains presynaptic glutamate release by regulating calcium-activated potassium channels in excitatory neurons. Mutations cause autosomal recessive intellectual developmental disorder, affecting cognitive functions including memory and learning. The gene shows very low constraint against loss-of-function variants (pLI 0.0003), consistent with its recessive inheritance pattern where heterozygous carriers are typically unaffected.

Summary from RefSeq, OMIM, UniProt
Research Assistant →

Primary Disease Associations & Inheritance

Intellectual developmental disorder, autosomal recessive 2MIM #607417
AR
0
Active trials
203
Pubs (1 yr)
96
P/LP submissions
2%
P/LP missense
0.64
LOEUF
LOF
Mechanism· G2P
Clinical SummaryCRBN
🧬
Gene-Disease Validity (ClinGen)
intellectual disability · ARModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
92 unique Pathogenic / Likely Pathogenic· 97 VUS of 231 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.64LOEUF
pLI 0.000
Z-score 3.01
OE 0.39 (0.240.64)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.04Z-score
OE missense 1.01 (0.911.12)
241 obs / 239.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.39 (0.240.64)
00.351.4
Missense OE1.01 (0.911.12)
00.61.4
Synonymous OE1.23
01.21.6
LoF obs/exp: 11 / 28.3Missense obs/exp: 241 / 239.2Syn Z: -1.64

ClinVar Variant Classifications

231 submitted variants in ClinVar

Classification Summary

Pathogenic78
Likely Pathogenic14
VUS97
Likely Benign19
Benign9
Conflicting5
78
Pathogenic
14
Likely Pathogenic
97
VUS
19
Likely Benign
9
Benign
5
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
3
0
75
0
78
Likely Pathogenic
1
2
11
0
14
VUS
3
67
27
0
97
Likely Benign
0
4
2
13
19
Benign
0
0
4
5
9
Conflicting
5
Total77311918222

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

View in ClinVar →

Protein Context — Lollipop Plot

CRBN · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 5 results · since 2015Search PubMed ↗