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

Publication

PubMed ID:
1998338 14702039 17974005 16572171 15489334 2336361 9101289 21269460 24275569 25944712 1401056 8364576 8318997 7942842 7977370 8005583 7757089 7550234 8557261 9633815 11196105 11476670 11278491 20003495