STN1

Chr 10AR

STN1 subunit of CST complex

Also known as: AAF-44, AAF44, CRMCC2, OBFC1, RPA-32, bA541N10.2

OBFC1 and C17ORF68 (MIM 613129) are subunits of an alpha accessory factor (AAF) that stimulates the activity of DNA polymerase-alpha-primase (see MIM 176636), the enzyme that initiates DNA replication (Casteel et al., 2009 [PubMed 19119139]). OBFC1 also appears to function in a telomere-associated complex with C17ORF68 and TEN1 (C17ORF106; MIM 613130) (Miyake et al., 2009 [PubMed 19854130]).[supplied by OMIM, Nov 2009]

GeneReviewsOMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 1.221 OMIM phenotype
Clinical SummarySTN1
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Gene-Disease Validity (ClinGen)
cerebroretinal microangiopathy with calcifications and cysts 2 · ARModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📖
GeneReview available — STN1
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.22LOEUF
pLI 0.000
Z-score 0.78
OE 0.82 (0.561.22)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.39Z-score
OE missense 0.92 (0.821.04)
186 obs / 201.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.82 (0.561.22)
00.351.4
Missense OE?0.92 (0.821.04)
00.61.4
Synonymous OE?1.00
01.21.6
LoF obs/exp: 17 / 20.8Missense obs/exp: 186 / 201.5Syn Z: -0.03
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongSTN1-related cerebroretinal microangiopathy with calcifications and cystsLOFAR

This gene — mechanism propensity

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6161th %ile
GOF
0.4776th %ile
LOF
0.3066th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

STN1 · 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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