AMER1

Chr X

APC membrane recruitment protein 1

Also known as: FAM123B, OSCS, WTX

The protein encoded by this gene upregulates trancriptional activation by the Wilms tumor protein and interacts with many other proteins, including CTNNB1, APC, AXIN1, and AXIN2. Defects in this gene are a cause of osteopathia striata with cranial sclerosis (OSCS). [provided by RefSeq, May 2010]

ResearchGenerating clinical summary…
LOFmechanismLOEUF 0.37
Clinical SummaryAMER1
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Gene-Disease Validity (ClinGen)
osteopathia striata with cranial sclerosis · XLDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.85) — some intolerance to loss-of-function variants.
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint?
0.37LOEUF
pLI 0.854
Z-score 4.06
OE 0.18 (0.090.37)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint?
-0.57Z-score
OE missense 1.08 (1.001.16)
474 obs / 440.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.18 (0.090.37)
00.351.4
Missense OE?1.08 (1.001.16)
00.61.4
Synonymous OE?1.11
01.21.6
LoF obs/exp: 5 / 28.3Missense obs/exp: 474 / 440.4Syn Z: -1.15
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongAMER1-related osteopathia striata with cranial sclerosisLOFmonoallelic_X_heterozygous

This gene — mechanism propensity

DN
0.3694th %ile
GOF
0.4283th %ile
LOF
0.72top 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

AMER1 · 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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