Names & Taxonomy
- Uniprot ID:
- P16930
- Entry Name:
- FAAA_HUMAN
- Status:
- reviewed
- Protein Names:
- Fumarylacetoacetase (FAA) (EC 3.7.1.2) (Beta-diketonase) (Fumarylacetoacetate hydrolase)
- Gene Names:
- FAH
- Gene Names Primary:
- FAH
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 419
- Sequence:
- MSFIPVAEDSDFPIHNLPYGVFSTRGDPRPRIGVAIGDQILDLSIIKHLFTGPVLSKHQDVFNQPTLNSFMGLGQAAWKEARVFLQNLLSVSQARLRDDTELRKCAFISQASATMHLPATIGDYTDFYSSRQHATNVGIMFRDKENALMPNWLHLPVGYHGRASSVVVSGTPIRRPMGQMKPDDSKPPVYGACKLLDMELEMAFFVGPGNRLGEPIPISKAHEHIFGMVLMNDWSARDIQKWEYVPLGPFLGKSFGTTVSPWVVPMDALMPFAVPNPKQDPRPLPYLCHDEPYTFDINLSVNLKGEGMSQAATICKSNFKYMYWTMLQQLTHHSVNGCNLRPGDLLASGTISGPEPENFGSMLELSWKGTKPIDLGNGQTRKFLLDGDEVIITGYCQGDGYRIGFGQCAGKVLPALLPS
- Proteomes:
- UP000005640
Function
- Pathway:
- Amino-acid degradation; L-phenylalanine degradation; acetoacetate and fumarate from L-phenylalanine: step 6/6.
- Catalytic Activity:
- 4-fumarylacetoacetate + H(2)O = acetoacetate + fumarate.
- Cofactor:
- COFACTOR: Name=Ca(2+); Xref=ChEBI:CHEBI:29108;
- Active Site:
- ACT_SITE 133 133 Proton acceptor.
- Gene Ontology Go:
- cytosol
extracellular exosome
fumarylacetoacetase activity
metal ion binding
arginine catabolic process
cellular nitrogen compound metabolic process
L-phenylalanine catabolic process
small molecule metabolic process
tyrosine catabolic process - Gene Ontology Biological Process:
- arginine catabolic process
cellular nitrogen compound metabolic process
L-phenylalanine catabolic process
small molecule metabolic process
tyrosine catabolic process - Gene Ontology Molecular Function:
- fumarylacetoacetase activity
metal ion binding - Gene Ontology Cellular Component:
- cytosol
extracellular exosome - Keywords:
- Acetylation
Alternative splicing
Calcium
Complete proteome
Direct protein sequencing
Disease mutation
Hydrolase
Magnesium
Metal-binding
Phenylalanine catabolism
Phosphoprotein
Reference proteome
Tyrosine catabolism - Interacts With:
- Q6UY14-3; P60410; P26371; Q53GC0; P15884