MSANTD2

Chr 11

Myb/SANT DNA binding domain containing 2

Also known as: C11orf61

The protein functions as a Myb/SANT DNA-binding domain-containing protein involved in transcriptional regulation. Mutations cause autosomal recessive intellectual disability with seizures and microcephaly, typically presenting in early childhood. This gene is highly constrained against loss-of-function variants, suggesting that complete loss of protein function is likely pathogenic.

0
Active trials
0
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.37
LOEUF
LOF
Mechanism· predicted
Clinical SummaryMSANTD2
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.90). One damaged copy is likely sufficient to cause disease.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.37LOEUF
pLI 0.903
Z-score 3.61
OE 0.14 (0.070.37)
Highly constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.30Z-score
OE missense 0.60 (0.520.68)
155 obs / 259.1 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.14 (0.070.37)
00.351.4
Missense OE0.60 (0.520.68)
00.61.4
Synonymous OE0.82
01.21.6
LoF obs/exp: 3 / 20.7Missense obs/exp: 155 / 259.1Syn Z: 1.46
DN
0.3694th %ile
GOF
0.4381th %ile
LOF
0.74top 10%

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

LOFprediction above median · LOEUF 0.37

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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MSANTD2 · 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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