SEMA4A

Chr 1AR

semaphorin 4A

Also known as: CORD10, RP35, SEMAB, SEMB

This gene encodes a member of the semaphorin family of soluble and transmembrane proteins. Semaphorins are involved in numerous functions, including axon guidance, morphogenesis, carcinogenesis, and immunomodulation. The encoded protein is a single-pass type I membrane protein containing an immunoglobulin-like C2-type domain, a PSI domain and a sema domain. It inhibits axonal extension by providing local signals to specify territories inaccessible for growing axons. It is an activator of T-cell-mediated immunity and suppresses vascular endothelial growth factor (VEGF)-mediated endothelial cell migration and proliferation in vitro and angiogenesis in vivo. Mutations in this gene are associated with retinal degenerative diseases including retinitis pigmentosa type 35 (RP35) and cone-rod dystrophy type 10 (CORD10). Multiple alternatively spliced transcript variants encoding different isoforms have been identified.[provided by RefSeq, Sep 2010]

GeneReviewsOMIMResearchGenerating clinical summary…
MultiplemechanismARLOEUF 0.642 OMIM phenotypes
Clinical SummarySEMA4A
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Gene-Disease Validity (ClinGen)
Lynch syndrome · ADDisputed

Disputed — evidence questions this relationship

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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ClinVar Variants
4 unique Pathogenic / Likely Pathogenic· 402 VUS of 690 total submissions
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GeneReview available — SEMA4A
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?
0.64LOEUF
pLI 0.000
Z-score 3.19
OE 0.41 (0.270.64)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.22Z-score
OE missense 0.97 (0.891.05)
412 obs / 424.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.41 (0.270.64)
00.351.4
Missense OE?0.97 (0.891.05)
00.61.4
Synonymous OE?0.96
01.21.6
LoF obs/exp: 14 / 34.1Missense obs/exp: 412 / 424.9Syn Z: 0.39
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedSEMA4A-related retinitis pigmentosaOTHERAR

This gene — mechanism propensity

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

DN
0.6840th %ile
GOF
0.6735th %ile
LOF
0.2678th %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

690 submitted variants in ClinVar

Classification Summary

Pathogenic3
Likely Pathogenic1
VUS402
Likely Benign220
Benign12
Conflicting37
3
Pathogenic
1
Likely Pathogenic
402
VUS
220
Likely Benign
12
Benign
37
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
2
1
0
0
3
Likely Pathogenic
0
1
0
0
1
VUS
29
339
28
6
402
Likely Benign
0
6
83
131
220
Benign
0
3
8
1
12
Conflicting
37
Total31350119138675

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

19 pathogenic / likely-pathogenic (of 32) ClinVar copy-number / structural variants overlap SEMA4A — these span large chromosomal regions, not the gene specifically, and are excluded from the counts above. Explore in CNV tools →

Protein Context — Lollipop Plot

SEMA4A · 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.

Search ClinicalTrials.gov →