ARRB2

Chr 17

arrestin beta 2

Also known as: ARB2, ARR2, BARR2

Arrestin beta 2 regulates G-protein coupled receptor signaling by mediating receptor desensitization, internalization, and serves as a signaling scaffold for MAPK and other pathways in the central nervous system and peripheral tissues. Mutations cause neurodevelopmental disorder with epilepsy, cataracts, and feeding difficulties, inherited in an autosomal recessive pattern. The gene shows significant constraint against loss-of-function variants (LOEUF 0.444), indicating intolerance to complete protein loss.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 0.44
Clinical SummaryARRB2
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.51) — some intolerance to loss-of-function variants.
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ClinVar Variants
22 unique Pathogenic / Likely Pathogenic· 52 VUS of 105 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.44LOEUF
pLI 0.507
Z-score 3.56
OE 0.21 (0.110.44)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.38Z-score
OE missense 0.58 (0.510.67)
150 obs / 257.7 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.21 (0.110.44)
00.351.4
Missense OE0.58 (0.510.67)
00.61.4
Synonymous OE0.89
01.21.6
LoF obs/exp: 5 / 23.7Missense obs/exp: 150 / 257.7Syn Z: 0.88
DN
0.7035th %ile
GOF
0.72top 25%
LOF
0.65top 25%

This gene has evidence for multiple mechanisms of pathogenicity (loss-of-function, gain-of-function and dominant-negative). The Badonyi & Marsh model scores gain-of-function highest among its predictions, but genomic evidence (constraint, ClinVar variant spectrum, and literature) most strongly supports loss-of-function (haploinsufficiency). Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

LOFprediction above median · LOEUF 0.44
GOFprediction above median
DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

105 submitted variants in ClinVar

Classification Summary

Pathogenic21
Likely Pathogenic1
VUS52
Likely Benign3
Benign2
21
Pathogenic
1
Likely Pathogenic
52
VUS
3
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
21
0
21
Likely Pathogenic
0
0
1
0
1
VUS
0
43
9
0
52
Likely Benign
0
2
1
0
3
Benign
0
0
0
2
2
Total04532279

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

View in ClinVar →

Protein Context — Lollipop Plot

ARRB2 · 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
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