FGF17

Chr 8AD

fibroblast growth factor 17

Also known as: FGF-13, FGF-17, HH20

This gene encodes fibroblast growth factor 17, which regulates embryonic brain development and patterning and is required for normal brain development. Mutations cause autosomal dominant hypogonadotropic hypogonadism with or without anosmia (Kallmann syndrome), affecting reproductive hormone regulation and sometimes sense of smell. The gene is highly constrained against loss-of-function variants (pLI 0.96), indicating that such variants are likely to be pathogenic.

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

Hypogonadotropic hypogonadism 20 with or without anosmiaMIM #615270
AD
0
Active trials
10
Pubs (1 yr)
82
P/LP submissions
1%
P/LP missense
0.30
LOEUF· LoF intol.
Mechanism
Clinical SummaryFGF17
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.96). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
82 unique Pathogenic / Likely Pathogenic· 38 VUS of 157 total submissions
📖
GeneReview available — FGF17
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.30LOEUF
pLI 0.956
Z-score 2.90
OE 0.00 (0.000.30)
Highly constrained

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

Missense Constraint
1.85Z-score
OE missense 0.58 (0.490.69)
89 obs / 153.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.000.30)
00.351.4
Missense OE0.58 (0.490.69)
00.61.4
Synonymous OE1.03
01.21.6
LoF obs/exp: 0 / 9.8Missense obs/exp: 89 / 153.7Syn Z: -0.21

ClinVar Variant Classifications

157 submitted variants in ClinVar

Classification Summary

Pathogenic79
Likely Pathogenic3
VUS38
Likely Benign23
Benign11
79
Pathogenic
3
Likely Pathogenic
38
VUS
23
Likely Benign
11
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
1
78
0
79
Likely Pathogenic
0
0
3
0
3
VUS
1
24
13
0
38
Likely Benign
0
1
10
12
23
Benign
0
0
8
3
11
Total12611215154

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

View in ClinVar →

Protein Context — Lollipop Plot

FGF17 · protein map & ClinVar variants

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

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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