SWI5

Chr 9

SWI5 homologous recombination repair protein

Also known as: C9orf119, SAE3

The protein is a component of the SWI5-SFR1 complex that functions in double-strand DNA break repair via homologous recombination. Mutations cause autosomal recessive severe combined immunodeficiency with microcephaly, growth retardation, and sensitivity to ionizing radiation. The gene shows low constraint to loss-of-function variation in the general population.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.10
Clinical SummarySWI5
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
1.10LOEUF
pLI 0.001
Z-score 1.34
OE 0.56 (0.301.10)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.97Z-score
OE missense 1.23 (1.091.40)
168 obs / 136.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.56 (0.301.10)
00.351.4
Missense OE1.23 (1.091.40)
00.61.4
Synonymous OE1.39
01.21.6
LoF obs/exp: 6 / 10.8Missense obs/exp: 168 / 136.3Syn Z: -2.30
DN
0.7325th %ile
GOF
0.4776th %ile
LOF
0.4234th %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

SWI5 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC