ABTB2

Chr 11

ankyrin repeat and BTB domain containing 2

Also known as: ABTB2A, BTBD22, CCA3

ABTB2 encodes a protein that enables protein heterodimerization and may be involved in hepatocyte growth initiation. The gene is highly constrained against loss-of-function variation (LOEUF 0.464), suggesting mutations would likely cause severe developmental phenotypes. However, no specific disease associations or inheritance patterns have been established for ABTB2 mutations in humans.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
2
Pubs (1 yr)
19
P/LP submissions
0%
P/LP missense
0.46
LOEUF
Mechanism
Clinical SummaryABTB2
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.29) despite low pLI — interpret in context.
📋
ClinVar Variants
19 unique Pathogenic / Likely Pathogenic· 175 VUS of 221 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.46LOEUF
pLI 0.005
Z-score 4.38
OE 0.29 (0.190.46)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.23Z-score
OE missense 0.86 (0.800.92)
516 obs / 600.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.29 (0.190.46)
00.351.4
Missense OE0.86 (0.800.92)
00.61.4
Synonymous OE0.93
01.21.6
LoF obs/exp: 13 / 44.5Missense obs/exp: 516 / 600.6Syn Z: 0.94

ClinVar Variant Classifications

221 submitted variants in ClinVar

Classification Summary

Pathogenic19
VUS175
Likely Benign2
19
Pathogenic
175
VUS
2
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
19
0
19
Likely Pathogenic
0
0
0
0
0
VUS
0
167
8
0
175
Likely Benign
0
2
0
0
2
Benign
0
0
0
0
0
Total0169270196

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

View in ClinVar →

Protein Context — Lollipop Plot

ABTB2 · 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 ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC