SYCE3

Chr 22

synaptonemal complex central element protein 3

Also known as: C22orf41, THEG2

SYCE3 encodes a major component of the synaptonemal complex central element that is required for proper chromosome pairing and recombination during meiosis. Mutations cause male infertility due to impaired spermatogenesis and abnormal testis development, with autosomal recessive inheritance reported in the literature. The gene shows intermediate constraint against loss-of-function variants (pLI 0.60, LOEUF 1.02).

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 1.02
Clinical SummarySYCE3
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.60) — some intolerance to loss-of-function variants.
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ClinVar Variants
146 unique Pathogenic / Likely Pathogenic· 26 VUS of 180 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
1.02LOEUF
pLI 0.600
Z-score 1.57
OE 0.00 (0.001.02)
Moderately constrained

Highly tolerant — LoF variants common in population

Missense Constraint
0.74Z-score
OE missense 0.71 (0.540.94)
36 obs / 50.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.001.02)
00.351.4
Missense OE0.71 (0.540.94)
00.61.4
Synonymous OE0.78
01.21.6
LoF obs/exp: 0 / 2.9Missense obs/exp: 36 / 50.8Syn Z: 0.74
DN
0.6840th %ile
GOF
0.79top 25%
LOF
0.3162th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
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

180 submitted variants in ClinVar

Classification Summary

Pathogenic145
Likely Pathogenic1
VUS26
Likely Benign1
Benign3
145
Pathogenic
1
Likely Pathogenic
26
VUS
1
Likely Benign
3
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
145
Likely Pathogenic
1
VUS
26
Likely Benign
1
Benign
3
Total176

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

SYCE3 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC