Gene

fah-1

Species
Caenorhabditis elegans
Symbol
fah-1
Name
FumarylAcetoacetate Hydrolase 1
Synonyms
  • CELE_K10C2.4
  • K10C2.4
Biotype
protein coding gene
Automated Description
Predicted to enable fumarylacetoacetase activity. Predicted to be involved in L-phenylalanine catabolic process; homogentisate catabolic process; and tyrosine catabolic process. Is expressed in hypodermis and intestine. Used to study tyrosinemia type I. Human ortholog(s) of this gene implicated in tyrosinemia type I. Orthologous to human FAH (fumarylacetoacetate hydrolase).
WB Description
fah-1 encodes a putative fumarylacetoacetate hydrolase, orthologous to human FAH (OMIM:276700, mutated in type I tyrosinemia), that is required for tyrosine metabolism, resistance to oxidative and protein-folding stresses, and more generally for normally rapid growth, normal locomotion, fertility, and viability; fah-1 is expressed in hypodermis and intestine; the intestines of fah-1(RNAi) animals atrophy rapidly, and show an abnormally active oxidative stress response (seen via gst-4::GFP) and ER stress response (seen via hsp-4::GFP); while RNAi against enzymes upstream of fah-1 suppresses the fah-1(RNAi) phenotype, excess dietary tyrosine or homogentisic acid enhances it, and exogenous succinylacetone (SA) mimics it, consistent with the hypothesis that blocked SA metabolism is toxic in fah-1(RNAi) animals.
Cross References
Additional Information
Literature

Orthology

Gene tree
PANTHER:PTHR43069
Links to orthology data in JBrowse by filter level: Stringent,  Moderate,  No filter,  Best and Best Reverse

Paralogy

Function - GO Annotations

Pathways

No data available

Phenotypes

Primary Sources
None
Other Sources
None

Disease Associations

Cases where the expected disease association was NOT found
Cell color indicative of annotation volume

Transgenic Alleles

Models

Sequence Feature Viewer

Genome location
Assembly version
WBcel235
Viewer Help
6.4468M6.4470M6.4472M6.4474M6.4476M6.4478M6.4480M6.4482M6.4484M6.4486M6.4488M

Sequence Details

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Expression

Primary Sources
Other Sources
Cell color indicative of annotation volume; red slash indicates species lacks structure or developmental stage.

Molecular Interactions

Genetic Interactions