ZSWIM7

Chr 17AR

zinc finger SWIM-type containing 7

Also known as: ODG10, SPGF71, SWS1

ZSWIM7 encodes a zinc-binding protein required for homologous recombination repair of DNA double-strand breaks and meiotic progression. Biallelic mutations cause autosomal recessive ovarian dysgenesis and male spermatogenic failure, presenting as reproductive disorders affecting fertility. The gene shows tolerance to loss-of-function variants in the general population (pLI 0.0009), consistent with recessive inheritance where heterozygous carriers are typically unaffected.

OMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismARLOEUF 1.212 OMIM phenotypes
Clinical SummaryZSWIM7
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.21LOEUF
pLI 0.001
Z-score 1.13
OE 0.61 (0.331.21)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.33Z-score
OE missense 0.89 (0.731.09)
67 obs / 75.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.61 (0.331.21)
00.351.4
Missense OE0.89 (0.731.09)
00.61.4
Synonymous OE0.84
01.21.6
LoF obs/exp: 6 / 9.8Missense obs/exp: 67 / 75.1Syn Z: 0.67
DN
0.6260th %ile
GOF
0.6150th %ile
LOF
0.2190th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ZSWIM7 · 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.

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Clinical Literature
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