SRSF6

Chr 20

serine and arginine rich splicing factor 6

Also known as: B52, HEL-S-91, SFRS6, SRP55

SRSF6 encodes a splicing factor that regulates constitutive mRNA splicing and modulates alternative splice site selection, including for MAPT/Tau and other transcripts involved in cellular differentiation and wound healing. Mutations cause autosomal dominant neurodevelopmental disorders with intellectual disability and developmental delay. The gene is highly constrained against loss-of-function variants, indicating intolerance to protein disruption.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.37
Clinical SummarySRSF6
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.91). One damaged copy is likely sufficient to cause disease.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.37LOEUF
pLI 0.911
Z-score 3.64
OE 0.14 (0.060.37)
Highly constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.10Z-score
OE missense 0.79 (0.700.90)
171 obs / 216.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.14 (0.060.37)
00.351.4
Missense OE0.79 (0.700.90)
00.61.4
Synonymous OE1.28
01.21.6
LoF obs/exp: 3 / 21.0Missense obs/exp: 171 / 216.5Syn Z: -1.86
DN
0.5771th %ile
GOF
0.5169th %ile
LOF
0.71top 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

SRSF6 · 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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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗