AHCTF1

Chr 1

AT-hook containing transcription factor 1

Also known as: ELYS, MST108, MSTP108, TMBS62

The protein is required for nuclear pore complex assembly and completion of cytokinesis by binding to chromatin and recruiting nuclear pore subcomplexes. Mutations cause autosomal dominant neurodevelopmental disorder with language impairment and behavioral abnormalities. This gene is extremely intolerant to loss-of-function variants, indicating that even heterozygous disruption can cause disease.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.13
Clinical SummaryAHCTF1
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.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.13LOEUF
pLI 1.000
Z-score 9.11
OE 0.07 (0.040.13)
Highly constrained

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

Missense Constraint
-0.38Z-score
OE missense 1.03 (0.981.08)
1176 obs / 1139.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.07 (0.040.13)
00.351.4
Missense OE1.03 (0.981.08)
00.61.4
Synonymous OE1.01
01.21.6
LoF obs/exp: 8 / 112.1Missense obs/exp: 1176 / 1139.8Syn Z: -0.12
DN
0.2599th %ile
GOF
0.14100th %ile
LOF
0.83top 5%

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

LOFprediction above median · LOEUF 0.13

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

AHCTF1 · 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
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗