SPMIP2

Chr 4

sperm microtubule inner protein 2

Also known as: C4orf45

The protein functions as a splicing regulatory factor that modulates alternative splicing of pre-mRNA transcripts. Mutations cause autosomal recessive intellectual disability with variable features including developmental delay, seizures, and behavioral abnormalities. This gene shows minimal constraint against loss-of-function variants, suggesting haploinsufficiency is well-tolerated.

0
Active trials
2
Pubs (1 yr)
36
P/LP submissions
0%
P/LP missense
1.59
LOEUF
Mechanism
Clinical SummarySPMIP2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
36 unique Pathogenic / Likely Pathogenic· 16 VUS of 58 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.59LOEUF
pLI 0.000
Z-score 0.14
OE 0.95 (0.591.59)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.09Z-score
OE missense 1.03 (0.871.22)
93 obs / 90.6 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.95 (0.591.59)
00.351.4
Missense OE1.03 (0.871.22)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 10 / 10.5Missense obs/exp: 93 / 90.6Syn Z: -0.25

ClinVar Variant Classifications

58 submitted variants in ClinVar

Classification Summary

Pathogenic33
Likely Pathogenic3
VUS16
Likely Benign1
33
Pathogenic
3
Likely Pathogenic
16
VUS
1
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
33
0
33
Likely Pathogenic
0
0
3
0
3
VUS
0
9
7
0
16
Likely Benign
0
0
0
1
1
Benign
0
0
0
0
0
Total0943153

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

View in ClinVar →

Protein Context — Lollipop Plot

SPMIP2 · 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 →
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 2 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found