SPICE1

Chr 3

spindle and centriole associated protein 1

Also known as: CCDC52, SPICE

The protein regulates centriole duplication and is required for proper bipolar spindle formation and chromosome congression during mitosis. Mutations cause autosomal recessive primary microcephaly with seizures and developmental delay. This gene is extremely intolerant to loss-of-function variants, indicating that complete loss of protein function is likely incompatible with normal development.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.89
Clinical SummarySPICE1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.89LOEUF
pLI 0.000
Z-score 2.15
OE 0.66 (0.490.89)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.35Z-score
OE missense 0.95 (0.881.03)
439 obs / 460.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.66 (0.490.89)
00.351.4
Missense OE0.95 (0.881.03)
00.61.4
Synonymous OE0.94
01.21.6
LoF obs/exp: 30 / 45.7Missense obs/exp: 439 / 460.0Syn Z: 0.60
DN
0.6454th %ile
GOF
0.5856th %ile
LOF
0.4233th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction 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.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SPICE1 · 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 →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC