FGFR4

Chr 5

fibroblast growth factor receptor 4

Also known as: CD334, JTK2, TKF

The FGFR4 protein is a tyrosine kinase that functions as a cell surface receptor for fibroblast growth factors and regulates cell proliferation, differentiation, migration, lipid metabolism, bile acid biosynthesis, and phosphate homeostasis. Mutations in this gene are associated with cancer progression and metastasis through aberrant signaling pathways. This gene shows low constraint against loss-of-function variants (LOEUF 0.87), suggesting tolerance to protein-truncating mutations.

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

{Cancer progression/metastasis}
3
Active trials
163
Pubs (1 yr)
62
P/LP submissions
0%
P/LP missense
0.87
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryFGFR4
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
60 unique Pathogenic / Likely Pathogenic· 104 VUS of 226 total submissions
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Clinical Trials
3 active or recruiting trials — potential therapeutic options may be available
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GeneReview available — FGFR4
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.87LOEUF
pLI 0.000
Z-score 2.24
OE 0.63 (0.460.87)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.19Z-score
OE missense 0.85 (0.790.92)
451 obs / 527.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.63 (0.460.87)
00.351.4
Missense OE0.85 (0.790.92)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 26 / 41.5Missense obs/exp: 451 / 527.6Syn Z: -0.69
DN
0.80top 25%
GOF
0.83top 10%
LOF
0.2679th %ile

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

GOFprediction above median
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

226 submitted variants in ClinVar

Classification Summary

Pathogenic56
Likely Pathogenic4
VUS104
Likely Benign17
Benign10
Conflicting1
56
Pathogenic
4
Likely Pathogenic
104
VUS
17
Likely Benign
10
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
56
0
56
Likely Pathogenic
0
0
4
0
4
VUS
1
98
4
1
104
Likely Benign
0
5
3
9
17
Benign
0
3
3
4
10
Conflicting
1
Total11067014192

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

View in ClinVar →

Protein Context — Lollipop Plot

FGFR4 · protein map & ClinVar variants

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

Clinical Literature
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