SPAG1

Chr 8AR

sperm associated antigen 1

Also known as: CILD28, CT140, DNAAF13, HEL-S-268, HSD-3.8, SP75, TPIS

The correlation of anti-sperm antibodies with cases of unexplained infertility implicates a role for these antibodies in blocking fertilization. Improved diagnosis and treatment of immunologic infertility, as well as identification of proteins for targeted contraception, are dependent on the identification and characterization of relevant sperm antigens. The protein expressed by this gene is recognized by anti-sperm agglutinating antibodies from an infertile woman. Furthermore, immunization of female rats with the recombinant human protein reduced fertility. This protein localizes to the plasma membrane of germ cells in the testis and to the post-acrosomal plasma membrane of mature spermatozoa. Recombinant polypeptide binds GTP and exhibits GTPase activity. Thus, this protein may regulate GTP signal transduction pathways involved in spermatogenesis and fertilization. Two transcript variants of this gene encode the same protein. [provided by RefSeq, Jul 2008]

Primary Disease Associations & Inheritance

Ciliary dyskinesia, primary, 28MIM #615505
AR
0
Active trials
11
Pubs (1 yr)
79
P/LP submissions
0%
P/LP missense
0.76
LOEUF
LOF
Mechanism· G2P
Clinical SummarySPAG1
🧬
Gene-Disease Validity (ClinGen)
primary ciliary dyskinesia 28 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
66 unique Pathogenic / Likely Pathogenic· 195 VUS of 500 total submissions
📖
GeneReview available — SPAG1
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
0.76LOEUF
pLI 0.000
Z-score 2.75
OE 0.53 (0.370.76)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.91Z-score
OE missense 0.88 (0.810.95)
376 obs / 428.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.53 (0.370.76)
00.351.4
Missense OE0.88 (0.810.95)
00.61.4
Synonymous OE0.77
01.21.6
LoF obs/exp: 21 / 39.7Missense obs/exp: 376 / 428.9Syn Z: 2.20
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveSPAG1-related primary ciliary dyskinesia associated with defective outer and inner dynein armsLOFAR

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

DN
0.7229th %ile
GOF
0.6736th %ile
LOF
0.2189th %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

500 submitted variants in ClinVar

Classification Summary

Pathogenic52
Likely Pathogenic14
VUS195
Likely Benign186
Benign11
Conflicting10
52
Pathogenic
14
Likely Pathogenic
195
VUS
186
Likely Benign
11
Benign
10
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
23
0
29
0
52
Likely Pathogenic
12
0
2
0
14
VUS
3
171
20
1
195
Likely Benign
0
12
73
101
186
Benign
0
2
8
1
11
Conflicting
10
Total38185132103468

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

View in ClinVar →

Protein Context — Lollipop Plot

SPAG1 · 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 →
Clinical Literature
Open Research Assistant →