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Protein

Fumarylacetoacetase

Gene

Fah

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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

  • arginine catabolic process Source: MGI
  • L-phenylalanine catabolic process Source: UniProtKB-UniPathway
  • tyrosine catabolic process Source: UniProtKB-KW
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase

Keywords - Biological processi

Phenylalanine catabolism, Tyrosine catabolism

Keywords - Ligandi

Calcium, Magnesium, Metal-binding

Enzyme and pathway databases

BRENDAi3.7.1.2. 3474.
ReactomeiR-MMU-71182. Phenylalanine and tyrosine catabolism.
UniPathwayiUPA00139; 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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 7

Organism-specific databases

MGIiMGI:95482. Fah.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

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-2DPAGEP35505.

PTM databases

iPTMnetiP35505.
PhosphoSitePlusiP35505.
SwissPalmiP35505.

Expressioni

Tissue specificityi

Mainly in liver and kidney.

Gene expression databases

BgeeiENSMUSG00000030630.
CleanExiMM_FAH.
ExpressionAtlasiP35505. baseline and differential.
GenevisibleiP35505. MM.

Interactioni

Subunit structurei

Homodimer.2 Publications

Protein-protein interaction databases

BioGridi199589. 1 interactor.
IntActiP35505. 6 interactors.
MINTiMINT-1854354.
STRINGi10090.ENSMUSP00000032865.

Structurei

Secondary structure

1419
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi2 – 4Combined sources3
Turni14 – 16Combined sources3
Beta strandi19 – 23Combined sources5
Beta strandi31 – 36Combined sources6
Beta strandi39 – 42Combined sources4
Turni43 – 46Combined sources4
Helixi47 – 49Combined sources3
Turni53 – 57Combined sources5
Helixi59 – 63Combined sources5
Beta strandi64 – 67Combined sources4
Helixi68 – 73Combined sources6
Helixi75 – 89Combined sources15
Turni90 – 94Combined sources5
Helixi95 – 98Combined sources4
Helixi100 – 106Combined sources7
Beta strandi107 – 110Combined sources4
Beta strandi114 – 116Combined sources3
Beta strandi124 – 127Combined sources4
Helixi131 – 142Combined sources12
Helixi144 – 146Combined sources3
Turni150 – 154Combined sources5
Beta strandi158 – 160Combined sources3
Beta strandi166 – 168Combined sources3
Beta strandi177 – 180Combined sources4
Beta strandi189 – 192Combined sources4
Beta strandi199 – 206Combined sources8
Helixi218 – 221Combined sources4
Helixi222 – 224Combined sources3
Beta strandi225 – 232Combined sources8
Helixi237 – 243Combined sources7
Turni245 – 247Combined sources3
Helixi251 – 254Combined sources4
Beta strandi257 – 259Combined sources3
Beta strandi263 – 265Combined sources3
Helixi266 – 269Combined sources4
Helixi270 – 272Combined sources3
Helixi285 – 287Combined sources3
Beta strandi298 – 304Combined sources7
Beta strandi312 – 318Combined sources7
Helixi319 – 321Combined sources3
Helixi326 – 334Combined sources9
Beta strandi345 – 347Combined sources3
Helixi356 – 358Combined sources3
Helixi362 – 365Combined sources4
Turni366 – 370Combined sources5
Beta strandi373 – 376Combined sources4
Beta strandi380 – 383Combined sources4
Beta strandi389 – 397Combined sources9
Beta strandi402 – 413Combined sources12

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...

Miscellaneous databases

EvolutionaryTraceiP35505.

Family & Domainsi

Sequence similaritiesi

Belongs to the FAH family.Curated

Phylogenomic databases

eggNOGiKOG2843. Eukaryota.
COG0179. LUCA.
GeneTreeiENSGT00390000008646.
HOGENOMiHOG000256845.
HOVERGENiHBG001919.
InParanoidiP35505.
KOiK01555.
OMAiSKSNFKH.
OrthoDBiEOG091G00CM.
TreeFamiTF315211.

Family and domain databases

Gene3Di2.30.30.230. 1 hit.
3.90.850.10. 1 hit.
InterProiIPR005959. Fumarylacetoacetase.
IPR011234. Fumarylacetoacetase_C-rel.
IPR015377. Fumarylacetoacetase_N.
[Graphical view]
PANTHERiPTHR11820:SF1. PTHR11820:SF1. 2 hits.
PfamiPF01557. FAA_hydrolase. 1 hit.
PF09298. FAA_hydrolase_N. 1 hit.
[Graphical view]
SUPFAMiSSF56529. SSF56529. 1 hit.
SSF63433. SSF63433. 1 hit.
TIGRFAMsiTIGR01266. fum_ac_acetase. 1 hit.

Sequencei

Sequence statusi: Complete.

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

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

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.

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.
MINTiMINT-1854354.
STRINGi10090.ENSMUSP00000032865.

PTM databases

iPTMnetiP35505.
PhosphoSitePlusiP35505.
SwissPalmiP35505.

2D gel databases

SWISS-2DPAGEP35505.

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.
OMAiSKSNFKH.
OrthoDBiEOG091G00CM.
TreeFamiTF315211.

Enzyme and pathway databases

UniPathwayiUPA00139; UER00341.
BRENDAi3.7.1.2. 3474.
ReactomeiR-MMU-71182. Phenylalanine and tyrosine catabolism.

Miscellaneous databases

EvolutionaryTraceiP35505.
PROiP35505.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000030630.
CleanExiMM_FAH.
ExpressionAtlasiP35505. baseline and differential.
GenevisibleiP35505. MM.

Family and domain databases

Gene3Di2.30.30.230. 1 hit.
3.90.850.10. 1 hit.
InterProiIPR005959. Fumarylacetoacetase.
IPR011234. Fumarylacetoacetase_C-rel.
IPR015377. Fumarylacetoacetase_N.
[Graphical view]
PANTHERiPTHR11820:SF1. PTHR11820:SF1. 2 hits.
PfamiPF01557. FAA_hydrolase. 1 hit.
PF09298. FAA_hydrolase_N. 1 hit.
[Graphical view]
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 historyi
Integrated into UniProtKB/Swiss-Prot: June 1, 1994
Last sequence update: July 27, 2011
Last modified: November 2, 2016
This is version 142 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.