ODF1

Chr 8

outer dense fiber of sperm tails 1

Also known as: CT133, HSPB10, ODF, ODF27, ODFP, ODFPG, ODFPGA, ODFPGB

The ODF1 protein is the main component of outer dense fibers, cytoskeletal structures that surround the sperm tail axoneme and maintain elastic structure while protecting against shear forces during sperm transport. Mutations cause abnormal sperm morphology and male infertility with autosomal recessive inheritance. This gene is highly constrained against loss-of-function variants, suggesting complete loss of function is likely incompatible with normal development.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
1
Pubs (1 yr)
41
P/LP submissions
0%
P/LP missense
0.27
LOEUF· LoF intol.
Multiple*
Mechanism· predicted
Clinical SummaryODF1
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.97). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
41 unique Pathogenic / Likely Pathogenic· 35 VUS of 81 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.27LOEUF
pLI 0.972
Z-score 3.08
OE 0.00 (0.000.27)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
0.68Z-score
OE missense 0.84 (0.730.98)
123 obs / 146.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.000.27)
00.351.4
Missense OE0.84 (0.730.98)
00.61.4
Synonymous OE0.93
01.21.6
LoF obs/exp: 0 / 11.0Missense obs/exp: 123 / 146.1Syn Z: 0.44
DN
0.5673th %ile
GOF
0.7029th %ile
LOF
0.60top 25%

This gene has evidence for multiple mechanisms of pathogenicity (loss-of-function and gain-of-function). The Badonyi & Marsh model scores gain-of-function highest among its predictions, but genomic evidence (constraint, ClinVar variant spectrum, and literature) most strongly supports loss-of-function (haploinsufficiency). Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

LOFLOEUF 0.27
GOFprediction 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. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

81 submitted variants in ClinVar

Classification Summary

Pathogenic39
Likely Pathogenic2
VUS35
Likely Benign1
Benign4
39
Pathogenic
2
Likely Pathogenic
35
VUS
1
Likely Benign
4
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
39
0
39
Likely Pathogenic
0
0
2
0
2
VUS
0
30
5
0
35
Likely Benign
0
0
1
0
1
Benign
2
0
1
1
4
Total23048181

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

View in ClinVar →

Protein Context — Lollipop Plot

ODF1 · 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 →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC