PF4V1

Chr 4

platelet factor 4 variant 1

Also known as: CXCL4L1, CXCL4V1, PF4-ALT, PF4A, SCYB4V1

The protein is a chemokine that inhibits angiogenesis and endothelial cell chemotaxis. Mutations cause autosomal recessive primary familial brain calcification, a neurodegenerative disorder characterized by bilateral calcification of the basal ganglia and other brain regions. The gene shows low constraint against loss-of-function variants, which is consistent with the recessive inheritance pattern.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.91
Clinical SummaryPF4V1
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
1.91LOEUF
pLI 0.001
Z-score -0.53
OE 1.33 (0.591.91)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.05Z-score
OE missense 0.98 (0.801.22)
60 obs / 61.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.33 (0.591.91)
00.351.4
Missense OE0.98 (0.801.22)
00.61.4
Synonymous OE1.17
01.21.6
LoF obs/exp: 4 / 3.0Missense obs/exp: 60 / 61.1Syn Z: -0.70
DN
0.74top 25%
GOF
0.5660th %ile
LOF
0.1796th %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

PF4V1 · 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.

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Clinical Literature
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