ZBTB44

Chr 11

zinc finger and BTB domain containing 44

Also known as: BTBD15, HSPC063, ZNF851

This protein is predicted to function as a transcriptional regulator through DNA binding and zinc ion binding activities in the nucleus. The gene is highly constrained against loss-of-function mutations (pLI 1.0, LOEUF 0.14), suggesting that heterozygous mutations would likely cause neurodevelopmental disorders with autosomal dominant inheritance. However, the specific phenotypes associated with ZBTB44 mutations have not been well-characterized in the literature.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
4
Pubs (1 yr)
81
P/LP submissions
0%
P/LP missense
0.14
LOEUF· LoF intol.
LOF
Mechanism· predicted
Clinical SummaryZBTB44
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
81 unique Pathogenic / Likely Pathogenic· 46 VUS of 142 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.14LOEUF
pLI 0.999
Z-score 4.24
OE 0.00 (0.000.14)
Highly constrained

Among the most LoF-intolerant genes (~top 3%)

Missense Constraint
1.87Z-score
OE missense 0.67 (0.590.76)
166 obs / 249.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.000.14)
00.351.4
Missense OE0.67 (0.590.76)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 0 / 20.9Missense obs/exp: 166 / 249.2Syn Z: -0.36
DN
0.2798th %ile
GOF
0.2497th %ile
LOF
0.82top 5%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.14

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

142 submitted variants in ClinVar

Classification Summary

Pathogenic74
Likely Pathogenic7
VUS46
Likely Benign2
74
Pathogenic
7
Likely Pathogenic
46
VUS
2
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
74
0
74
Likely Pathogenic
0
0
7
0
7
VUS
0
45
1
0
46
Likely Benign
0
1
1
0
2
Benign
0
0
0
0
0
Total046830129

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

View in ClinVar →

Protein Context — Lollipop Plot

ZBTB44 · 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 →
Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗
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