FAH

Chr 15

fumarylacetoacetate hydrolase

Predicted to enable fumarylacetoacetase activity. Predicted to be involved in L-phenylalanine catabolic process; homogentisate catabolic process; and tyrosine catabolic process. Predicted to act upstream of or within arginine catabolic process. Located in extracellular exosome. Implicated in tyrosinemia type I. [provided by Alliance of Genome Resources, Apr 2025]

ResearchGenerating clinical summary…
LOFmechanismLOEUF 1.41
Clinical SummaryFAH
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Gene-Disease Validity (ClinGen)
tyrosinemia type I · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
6 active or recruiting trials — potential therapeutic options may be available
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.41LOEUF
pLI 0.000
Z-score -0.06
OE 1.01 (0.741.41)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.03Z-score
OE missense 1.01 (0.911.12)
244 obs / 242.6 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?1.01 (0.741.41)
00.351.4
Missense OE?1.01 (0.911.12)
00.61.4
Synonymous OE?1.08
01.21.6
LoF obs/exp: 25 / 24.7Missense obs/exp: 244 / 242.6Syn Z: -0.61

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

FAH · protein map & ClinVar variants

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