POSTN

Chr 13

periostin

Also known as: OSF-2, OSF2, PDLPOSTN, PN

The protein encoded by this gene is a secreted extracellular matrix protein that binds to integrins to support cell adhesion and migration, and enhances incorporation of proteins involved in connective tissue formation. Mutations cause autosomal recessive osteogenesis imperfecta type XV, characterized by bone fragility, skeletal deformities, and dental abnormalities. This gene shows minimal constraint against loss-of-function variants, consistent with a recessive inheritance pattern where heterozygous carriers are typically unaffected.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.72
Clinical SummaryPOSTN
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.72LOEUF
pLI 0.000
Z-score 3.00
OE 0.51 (0.360.72)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.85Z-score
OE missense 0.89 (0.810.96)
393 obs / 443.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.51 (0.360.72)
00.351.4
Missense OE0.89 (0.810.96)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 22 / 43.3Missense obs/exp: 393 / 443.5Syn Z: -0.62
DN
0.73top 25%
GOF
0.6151th %ile
LOF
0.3163th %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

POSTN · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold
Clinical Literature
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