SPMIP3

Chr 1

sperm microtubule inner protein 3

Also known as: C1orf100

This gene encodes a protein involved in sperm motility and flagellar function. Mutations cause primary ciliary dyskinesia with infertility in males, following an autosomal recessive inheritance pattern. The gene shows low constraint against loss-of-function variants, which is consistent with the recessive disease mechanism.

LOEUF 1.54
Clinical SummarySPMIP3
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
93 unique Pathogenic / Likely Pathogenic· 11 VUS of 110 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.54LOEUF
pLI 0.000
Z-score 0.20
OE 0.94 (0.591.54)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.18Z-score
OE missense 1.05 (0.891.25)
91 obs / 86.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.94 (0.591.54)
00.351.4
Missense OE1.05 (0.891.25)
00.61.4
Synonymous OE0.96
01.21.6
LoF obs/exp: 11 / 11.8Missense obs/exp: 91 / 86.4Syn Z: 0.19

ClinVar Variant Classifications

110 submitted variants in ClinVar

Classification Summary

Pathogenic86
Likely Pathogenic7
VUS11
Likely Benign1
86
Pathogenic
7
Likely Pathogenic
11
VUS
1
Likely Benign

Curated Variants Distribution

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

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
86
Likely Pathogenic
7
VUS
11
Likely Benign
1
Benign
0
Total105

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

SPMIP3 · 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 →
Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 1 full-text resultsSearch PubTator3 ↗
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

No open access results found