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Protein

Fumarylacetoacetase

Gene

Fah

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Catalytic activityi

4-fumarylacetoacetate + H2O = acetoacetate + fumarate.2 Publications

Cofactori

Protein has several cofactor binding sites:

Pathwayi: L-phenylalanine degradation

This protein is involved in step 6 of the subpathway that synthesizes acetoacetate and fumarate from L-phenylalanine.
Proteins known to be involved in the 6 steps of the subpathway in this organism are:
  1. Phenylalanine-4-hydroxylase (Pah)
  2. Tyrosine aminotransferase (Tat)
  3. 4-hydroxyphenylpyruvate dioxygenase (Hpd)
  4. Homogentisate 1,2-dioxygenase (Hgd)
  5. Maleylacetoacetate isomerase (Gstz1)
  6. Fumarylacetoacetase (Fah)
This subpathway is part of the pathway L-phenylalanine degradation, which is itself part of Amino-acid degradation.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes acetoacetate and fumarate from L-phenylalanine, the pathway L-phenylalanine degradation and in Amino-acid degradation.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi126Calcium2 Publications1
Binding sitei128Substrate1 Publication1
Active sitei133Proton acceptor1 Publication1
Binding sitei142Substrate1 Publication1
Metal bindingi199Calcium2 Publications1
Metal bindingi201Calcium2 Publications1
Metal bindingi233Calcium2 Publications1
Metal bindingi233Magnesium1 Publication1
Binding sitei240Substrate1 Publication1
Binding sitei244Substrate1 Publication1
Metal bindingi253Magnesium1 Publication1
Metal bindingi257Magnesium1 Publication1
Binding sitei350Substrate1 Publication1

GO - Molecular functioni

  • fumarylacetoacetase activity Source: MGI
  • metal ion binding Source: UniProtKB-KW

GO - Biological processi

Keywordsi

Molecular functionHydrolase
Biological processPhenylalanine catabolism, Tyrosine catabolism
LigandCalcium, Magnesium, Metal-binding

Enzyme and pathway databases

BRENDAi3.7.1.2 3474
ReactomeiR-MMU-71182 Phenylalanine and tyrosine catabolism
UniPathwayi
UPA00139;UER00341

Names & Taxonomyi

Protein namesi
Recommended name:
Fumarylacetoacetase (EC:3.7.1.22 Publications)
Short name:
FAA
Alternative name(s):
Beta-diketonase
Fumarylacetoacetate hydrolase
Gene namesi
Name:Fah
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 7

Organism-specific databases

MGIiMGI:95482 Fah

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi201E → G: Decrease in activity. 1 Publication1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedBy similarity
ChainiPRO_00001568262 – 419FumarylacetoacetaseAdd BLAST418

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylserineBy similarity1
Modified residuei84PhosphoserineCombined sources1
Modified residuei92PhosphoserineCombined sources1
Modified residuei309PhosphoserineBy similarity1
Modified residuei417PhosphoserineBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP35505
MaxQBiP35505
PaxDbiP35505
PeptideAtlasiP35505
PRIDEiP35505

2D gel databases

SWISS-2DPAGEiP35505

PTM databases

iPTMnetiP35505
PhosphoSitePlusiP35505
SwissPalmiP35505

Expressioni

Tissue specificityi

Mainly in liver and kidney.

Gene expression databases

BgeeiENSMUSG00000030630 Expressed in 273 organ(s), highest expression level in liver
CleanExiMM_FAH
ExpressionAtlasiP35505 baseline and differential
GenevisibleiP35505 MM

Interactioni

Subunit structurei

Homodimer.2 Publications

Protein-protein interaction databases

BioGridi199589, 1 interactor
IntActiP35505, 6 interactors
MINTiP35505
STRINGi10090.ENSMUSP00000032865

Structurei

Secondary structure

1419
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP35505
SMRiP35505
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP35505

Family & Domainsi

Sequence similaritiesi

Belongs to the FAH family.Curated

Phylogenomic databases

eggNOGiKOG2843 Eukaryota
COG0179 LUCA
GeneTreeiENSGT00390000008646
HOGENOMiHOG000256845
HOVERGENiHBG001919
InParanoidiP35505
KOiK01555
OMAiPNWLHIP
OrthoDBiEOG091G00CM
TreeFamiTF315211

Family and domain databases

Gene3Di2.30.30.230, 1 hit
3.90.850.10, 1 hit
InterProiView protein in InterPro
IPR005959 Fumarylacetoacetase
IPR011234 Fumarylacetoacetase-like_C
IPR036663 Fumarylacetoacetase_C_sf
IPR015377 Fumarylacetoacetase_N
IPR036462 Fumarylacetoacetase_N_sf
PANTHERiPTHR43069 PTHR43069, 1 hit
PfamiView protein in Pfam
PF01557 FAA_hydrolase, 1 hit
PF09298 FAA_hydrolase_N, 1 hit
SUPFAMiSSF56529 SSF56529, 1 hit
SSF63433 SSF63433, 1 hit
TIGRFAMsiTIGR01266 fum_ac_acetase, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

P35505-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MSFIPVAEDS DFPIQNLPYG VFSTQSNPKP RIGVAIGDQI LDLSVIKHLF
60 70 80 90 100
TGPALSKHQH VFDETTLNNF MGLGQAAWKE ARASLQNLLS ASQARLRDDK
110 120 130 140 150
ELRQRAFTSQ ASATMHLPAT IGDYTDFYSS RQHATNVGIM FRGKENALLP
160 170 180 190 200
NWLHLPVGYH GRASSIVVSG TPIRRPMGQM RPDNSKPPVY GACRLLDMEL
210 220 230 240 250
EMAFFVGPGN RFGEPIPISK AHEHIFGMVL MNDWSARDIQ QWEYVPLGPF
260 270 280 290 300
LGKSFGTTIS PWVVPMDALM PFVVPNPKQD PKPLPYLCHS QPYTFDINLS
310 320 330 340 350
VSLKGEGMSQ AATICRSNFK HMYWTMLQQL THHSVNGCNL RPGDLLASGT
360 370 380 390 400
ISGSDPESFG SMLELSWKGT KAIDVEQGQT RTFLLDGDEV IITGHCQGDG
410
YRVGFGQCAG KVLPALSPA
Length:419
Mass (Da):46,176
Last modified:July 27, 2011 - v2
Checksum:i771C247377B59102
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
D3Z2R9D3Z2R9_MOUSE
Fumarylacetoacetase
Fah
141Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti129 – 130SS → PF in CAA77819 (PubMed:1524868).Curated2
Sequence conflicti307G → R no nucleotide entry (PubMed:1644288).Curated1
Sequence conflicti376E → G in AAA37591 (PubMed:1741389).Curated1
Sequence conflicti376E → G in AAH10767 (PubMed:15489334).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M84145 mRNA Translation: AAA37591.1
Z11774 mRNA Translation: CAA77819.1
AK143759 mRNA Translation: BAE25527.1
AK158808 mRNA Translation: BAE34676.1
BC010767 mRNA Translation: AAH10767.1
CCDSiCCDS21418.1
PIRiA40219
A56825
RefSeqiNP_034306.2, NM_010176.4
UniGeneiMm.3798

Genome annotation databases

EnsembliENSMUST00000032865; ENSMUSP00000032865; ENSMUSG00000030630
GeneIDi14085
KEGGimmu:14085
UCSCiuc009iej.3 mouse

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M84145 mRNA Translation: AAA37591.1
Z11774 mRNA Translation: CAA77819.1
AK143759 mRNA Translation: BAE25527.1
AK158808 mRNA Translation: BAE34676.1
BC010767 mRNA Translation: AAH10767.1
CCDSiCCDS21418.1
PIRiA40219
A56825
RefSeqiNP_034306.2, NM_010176.4
UniGeneiMm.3798

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1HYOX-ray1.30A/B1-419[»]
1QCNX-ray1.90A/B1-419[»]
1QCOX-ray1.90A/B1-419[»]
1QQJX-ray1.55A/B1-419[»]
2HZYX-ray1.35A/B1-419[»]
ProteinModelPortaliP35505
SMRiP35505
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi199589, 1 interactor
IntActiP35505, 6 interactors
MINTiP35505
STRINGi10090.ENSMUSP00000032865

PTM databases

iPTMnetiP35505
PhosphoSitePlusiP35505
SwissPalmiP35505

2D gel databases

SWISS-2DPAGEiP35505

Proteomic databases

EPDiP35505
MaxQBiP35505
PaxDbiP35505
PeptideAtlasiP35505
PRIDEiP35505

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000032865; ENSMUSP00000032865; ENSMUSG00000030630
GeneIDi14085
KEGGimmu:14085
UCSCiuc009iej.3 mouse

Organism-specific databases

CTDi2184
MGIiMGI:95482 Fah

Phylogenomic databases

eggNOGiKOG2843 Eukaryota
COG0179 LUCA
GeneTreeiENSGT00390000008646
HOGENOMiHOG000256845
HOVERGENiHBG001919
InParanoidiP35505
KOiK01555
OMAiPNWLHIP
OrthoDBiEOG091G00CM
TreeFamiTF315211

Enzyme and pathway databases

UniPathwayi
UPA00139;UER00341

BRENDAi3.7.1.2 3474
ReactomeiR-MMU-71182 Phenylalanine and tyrosine catabolism

Miscellaneous databases

EvolutionaryTraceiP35505
PROiPR:P35505
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000030630 Expressed in 273 organ(s), highest expression level in liver
CleanExiMM_FAH
ExpressionAtlasiP35505 baseline and differential
GenevisibleiP35505 MM

Family and domain databases

Gene3Di2.30.30.230, 1 hit
3.90.850.10, 1 hit
InterProiView protein in InterPro
IPR005959 Fumarylacetoacetase
IPR011234 Fumarylacetoacetase-like_C
IPR036663 Fumarylacetoacetase_C_sf
IPR015377 Fumarylacetoacetase_N
IPR036462 Fumarylacetoacetase_N_sf
PANTHERiPTHR43069 PTHR43069, 1 hit
PfamiView protein in Pfam
PF01557 FAA_hydrolase, 1 hit
PF09298 FAA_hydrolase_N, 1 hit
SUPFAMiSSF56529 SSF56529, 1 hit
SSF63433 SSF63433, 1 hit
TIGRFAMsiTIGR01266 fum_ac_acetase, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiFAAA_MOUSE
AccessioniPrimary (citable) accession number: P35505
Secondary accession number(s): Q3TY87, Q9QW65
Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 1, 1994
Last sequence update: July 27, 2011
Last modified: September 12, 2018
This is version 149 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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