MOSPD2

Chr X

motile sperm domain containing 2

The MOSPD2 protein mediates formation of contact sites between the endoplasmic reticulum and other organelles including endosomes, mitochondria, and Golgi, and participates in lipid droplet homeostasis. Mutations cause autosomal recessive intellectual disability with developmental delay, and the gene is highly constrained against loss-of-function variants (pLI 0.99, LOEUF 0.26). The condition primarily affects neurological development and cognitive function.

ResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.26
Clinical SummaryMOSPD2
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.99). One damaged copy is likely sufficient to cause disease.
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ClinVar Variants
68 unique Pathogenic / Likely Pathogenic· 34 VUS of 229 total submissions
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.26LOEUF
pLI 0.991
Z-score 3.78
OE 0.05 (0.020.26)
Highly constrained

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

Missense Constraint
1.66Z-score
OE missense 0.65 (0.560.76)
115 obs / 177.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.05 (0.020.26)
00.351.4
Missense OE0.65 (0.560.76)
00.61.4
Synonymous OE0.62
01.21.6
LoF obs/exp: 1 / 18.5Missense obs/exp: 115 / 177.2Syn Z: 2.32
DN
0.2698th %ile
GOF
0.4184th %ile
LOF
0.68top 10%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.26

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

229 submitted variants in ClinVar

Classification Summary

Pathogenic67
Likely Pathogenic1
VUS34
Likely Benign3
Benign2
67
Pathogenic
1
Likely Pathogenic
34
VUS
3
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
67
0
67
Likely Pathogenic
0
0
1
0
1
VUS
0
28
6
0
34
Likely Benign
0
1
1
1
3
Benign
0
0
1
1
2
Total029762107

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

View in ClinVar →

Protein Context — Lollipop Plot

MOSPD2 · 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 →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗