SALL3

Chr 18

spalt like transcription factor 3

Also known as: ZNF796

The protein is a zinc-finger transcription factor that regulates DNA methylation by binding to and inhibiting DNA methyltransferase 3 alpha (DNMT3A). Mutations cause autosomal dominant intellectual disability with developmental delay and distinctive facial features. The gene shows significant constraint against loss-of-function variants (LOEUF 0.44), reflecting its important role in embryonic development.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
1
Pubs (1 yr)
68
P/LP submissions
0%
P/LP missense
0.44
LOEUF
LOF
Mechanism· predicted
Clinical SummarySALL3
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.22) despite low pLI — interpret in context.
📋
ClinVar Variants
68 unique Pathogenic / Likely Pathogenic· 114 VUS of 199 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.44LOEUF
pLI 0.403
Z-score 3.74
OE 0.22 (0.120.44)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.31Z-score
OE missense 0.97 (0.911.03)
756 obs / 780.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.22 (0.120.44)
00.351.4
Missense OE0.97 (0.911.03)
00.61.4
Synonymous OE1.17
01.21.6
LoF obs/exp: 6 / 27.0Missense obs/exp: 756 / 780.4Syn Z: -2.66
DN
0.4686th %ile
GOF
0.3292th %ile
LOF
0.69top 10%

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

LOFprediction above median · LOEUF 0.44

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

199 submitted variants in ClinVar

Classification Summary

Pathogenic62
Likely Pathogenic6
VUS114
Likely Benign11
Benign1
62
Pathogenic
6
Likely Pathogenic
114
VUS
11
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
62
0
62
Likely Pathogenic
0
0
6
0
6
VUS
0
102
12
0
114
Likely Benign
0
6
3
2
11
Benign
0
0
0
1
1
Total0108833194

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

View in ClinVar →

Protein Context — Lollipop Plot

SALL3 · 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.

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Clinical Literature
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