FGF9

Chr 13AD

fibroblast growth factor 9

Also known as: FGF-9, GAF, HBFG-9, HBGF-9, SYNS3

The protein is a secreted fibroblast growth factor that regulates embryonic development, cell proliferation and differentiation, and plays a key role in glial cell development and neuronal cell survival. Mutations cause multiple synostoses syndrome 3, characterized by progressive fusion of joints and bones, with autosomal dominant inheritance. The gene is highly constrained against loss-of-function variants (pLI 0.95, LOEUF 0.32), indicating intolerance to protein-disrupting mutations.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismADLOEUF 0.321 OMIM phenotype
Clinical SummaryFGF9
🧬
Gene-Disease Validity (ClinGen)
multiple synostoses syndrome · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.95). One damaged copy is likely sufficient to cause disease.
📖
GeneReview available — FGF9
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.32LOEUF
pLI 0.947
Z-score 2.82
OE 0.00 (0.000.32)
Highly constrained

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

Missense Constraint
1.54Z-score
OE missense 0.60 (0.490.73)
70 obs / 116.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.000.32)
00.351.4
Missense OE0.60 (0.490.73)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 0 / 9.3Missense obs/exp: 70 / 116.9Syn Z: -0.01
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongFGF9-related multiple synostoses syndromeLOFAD
DN
0.3793th %ile
GOF
0.6541th %ile
LOF
0.66top 25%

This gene has evidence for multiple mechanisms of pathogenicity (loss-of-function and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to loss-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

LOFprediction above median · 1 literature citation · LOEUF 0.32
GOFprediction 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.

Literature Evidence

LOFImportantly, although other pro-osteogenic factors [Fgf-2, Fgf-18, and bone morphogenic protein 2 (Bmp-2)] still were present in Fgf-9(+/-) mice, they could not compensate for the haploinsufficiency of the Fgf-9 gene.PMID:20547837

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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

FGF9 · 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 →