ATF7IP

Chr 12

activating transcription factor 7 interacting protein

Also known as: AM, ATF-IP, ATF7IP1, MCAF, MCAF1, p621

ATF7IP encodes a nuclear protein that regulates gene expression by recruiting transcriptional complexes to chromatin and facilitating histone modifications, particularly H3K9 trimethylation for gene silencing. Mutations cause autosomal dominant neurodevelopmental disorder with intellectual disability, seizures, and behavioral abnormalities. This gene is highly constrained against loss-of-function variants (pLI 1.0, LOEUF 0.21), indicating that heterozygous mutations are likely pathogenic.

Summary from RefSeq, UniProt
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0
Active trials
14
Pubs (1 yr)
41
P/LP submissions
0%
P/LP missense
0.21
LOEUF· LoF intol.
LOF
Mechanism· predicted
Clinical SummaryATF7IP
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.
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ClinVar Variants
41 unique Pathogenic / Likely Pathogenic· 133 VUS of 220 total submissions
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GeneReview available — ATF7IP
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.21LOEUF
pLI 1.000
Z-score 5.85
OE 0.10 (0.050.21)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
1.78Z-score
OE missense 0.81 (0.750.87)
543 obs / 672.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.10 (0.050.21)
00.351.4
Missense OE0.81 (0.750.87)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 5 / 49.4Missense obs/exp: 543 / 672.7Syn Z: 0.12
DN
0.13100th %ile
GOF
0.09100th %ile
LOF
0.86top 5%

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

LOFprediction above median · LOEUF 0.21

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

220 submitted variants in ClinVar

Classification Summary

Pathogenic38
Likely Pathogenic3
VUS133
Likely Benign14
Benign8
38
Pathogenic
3
Likely Pathogenic
133
VUS
14
Likely Benign
8
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
38
0
38
Likely Pathogenic
0
0
3
0
3
VUS
0
130
3
0
133
Likely Benign
0
11
1
2
14
Benign
0
5
0
3
8
Total0146455196

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

View in ClinVar →

Protein Context — Lollipop Plot

ATF7IP · 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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