fumarylacetoacetase

Stable Identifier
R-OSA-1129390
Type
Protein [EntityWithAccessionedSequence]
Species
Oryza sativa
Synonyms
phys-ent-participant15034
Locations in the PathwayBrowser
Expand all
Summation

This is the final step of tyrosine degradation. Fumarylacetoacetase catalyzes the hydrolysis of 4-fumarylacetoacetate (4FAA) to form fumarate and acetoacetate. The enzyme is a homodimer, with each monomer binding one Ca(2+) and one Mg(2+) ion as catalytic cofactors, as shown in a mouse structure (Hsiang et al., 1972; Labelle et al. 1993; Bateman et al., 2001). FAH deficiency leads to tyrosinemia type I (TYRSN1, MIM:276700) characterized by 4FAA accumulation with severe metabolic effects (reviewed in Pitkänen et al., 2000; Scott, 2006).

External Reference Information
External Reference
UniProt:Q10B63 FAHD2
Gene Names
FAHD2, Os03g0829000, LOC_Os03g61330, OsJ_13211, OSJNBa0010E04.1, OSJNBb0027B08.34
Chain
transit peptide:1-30, chain:31-226
Participant Of
hasMember
Inferred To
AT3G16700 [mitochondrion] (Arabidopsis thaliana)
Zjn_sc00013.1.g09280.1.sm.mkhc [mitochondrion] (Zoysia japonica)
Vigun03g420700.v1.2 [mitochondrion] (Vigna unguiculata)
HUMLU_hap_000581F_002.g3.t1 [mitochondrion] (Humulus lupulus)
g20210 [mitochondrion] (Arabidopsis halleri)
gene-BT93_H0829 [mitochondrion] (Corymbia citriodora)
HORVU.MOREX.r3.4HG0342690 [mitochondrion] (Hordeum vulgare)
AMTR_s00183p00050040 [mitochondrion] (Amborella trichopoda)
TraesCS4B02G078600 [mitochondrion] (Triticum aestivum)
AET4Gv20163600 [mitochondrion] (Aegilops tauschii)
OfLcomp119860_c0_seq1 [mitochondrion] (Oryza officinalis)
SELMODRAFT_108260 [mitochondrion] (Selaginella moellendorffii)
TraesTSP4B01G082900 [mitochondrion] (Triticum spelta subsp. spelta)
ORUFI03G41420 [mitochondrion] (Oryza rufipogon)
PDK_30s984851g002 [mitochondrion] (Phoenix dactylifera)
GSBRNA2T00022769001 [mitochondrion] (Brassica napus)
NNU_14007-RA [mitochondrion] (Nelumbo nucifera)
A08p018110.1_BraROA [mitochondrion] (Brassica rapa)
TRIDC4AG037280 [mitochondrion] (Triticum dicoccoides)
GLYMA_17G028100 [mitochondrion] (Glycine max)
Migut.N00082.1 [mitochondrion] (Erythranthe guttata)
BVRB_3g067140 [mitochondrion] (Beta vulgaris)
CBR_g26355 [mitochondrion] (Chara braunii)
QL06p010180 [mitochondrion] (Quercus lobata)
Gasu_60660 [mitochondrion] (Galdieria sulphuraria)
MiLcomp139173_c1_seq1 [mitochondrion] (Oryza minuta)
OB03G46700 [mitochondrion] (Oryza brachyantha)
TCM_021244 [mitochondrion] (Theobroma cacao)
TRITD4Av1G177160 [mitochondrion] (Triticum turgidum)
Csa12g053610 [mitochondrion] (Camelina sativa)
OPUNC03G36770 [mitochondrion] (Oryza punctata)
Zjn_sc00014.1.g09180.1.sm.mkhc [mitochondrion] (Zoysia japonica)
TraesTSP4D01G081000 [mitochondrion] (Triticum spelta subsp. spelta)
HanXRQr2_Chr06g0251801 [mitochondrion] (Helianthus annuus)
TRIDC4BG012200 [mitochondrion] (Triticum dicoccoides)
Gasu_27240 [mitochondrion] (Galdieria sulphuraria)
GrLcomp98378_c1_seq2 [mitochondrion] (Oryza meyeriana var. granulata)
Ca_02966 [mitochondrion] (Cicer arietinum)
Manes.03G198200.v8.1 [mitochondrion] (Manihot esculenta)
Ma03_g25350 [mitochondrion] (Musa acuminata)
Csa15g021590 [mitochondrion] (Camelina sativa)
Tritim_EIv0.3_0838510 [mitochondrion] (Triticum timopheevii subsp. timopheevii)
OfLcomp6814_c0_seq2 [mitochondrion] (Oryza officinalis)
EUGRSUZ_C01303 [mitochondrion] (Eucalyptus grandis)
Vfaba.Hedin2.R1.4g194200 [mitochondrion] (Vicia faba var. faba)
Bo8g042180 [mitochondrion] (Brassica oleracea)
CMJ301C [mitochondrion] (Cyanidioschyzon merolae)
AUR62025637 [mitochondrion] (Chenopodium quinoa)
PH01000040G1720 [mitochondrion] (Phyllostachys edulis)
Aco000664.1.path1 [mitochondrion] (Ananas comosus)
Pp3c9_26130 [mitochondrion] (Physcomitrella patens)
gene-LSAT_3X24101 [mitochondrion] (Lactuca sativa)
TuG1812G0400000751.01 [mitochondrion] (Triticum urartu)
gene-BT93_C0755 [mitochondrion] (Corymbia citriodora)
PRUPE_1G125100 [mitochondrion] (Prunus persica)
mrna07034.1-v1.0-hybrid [mitochondrion] (Fragaria vesca)
FCD_00023334 [mitochondrion] (Ficus carica)
Csa11g034280 [mitochondrion] (Camelina sativa)
Vradi07g25080 [mitochondrion] (Vigna radiata)
orange1.1g032692m [mitochondrion] (Citrus sinensis)
BVRB_7g177710 [mitochondrion] (Beta vulgaris)
CEY00_Acc33090 [mitochondrion] (Actinidia chinensis)
itb13g22130 [mitochondrion] (Ipomoea triloba)
AuLcomp16350_c0_seq1 [mitochondrion] (Oryza australiensis)
arahy.Tifrunner.gnm2.ann2.Ah01g358900 [mitochondrion] (Arachis hypogaea)
EUGRSUZ_C01444 [mitochondrion] (Eucalyptus grandis)
CICLE_v10006277mg [mitochondrion] (Citrus clementina)
gene-CFP56_35953 [mitochondrion] (Quercus suber)
DCAR_023296 [mitochondrion] (Daucus carota)
fgenesh2_kg.3__1838__AT3G16700.1 [mitochondrion] (Arabidopsis lyrata)
CHC_T00010274001 [mitochondrion] (Chondrus crispus)
OE9A046022 [mitochondrion] (Olea europaea)
OE9A046022 [mitochondrion] (Olea europaea var. sylvestris)
Csa01g019380 [mitochondrion] (Camelina sativa)
g36862 [mitochondrion] (Salvia hispanica)
itb11g00050 [mitochondrion] (Ipomoea triloba)
C.cajan_04306 [mitochondrion] (Cajanus cajan)
HUMLU_pri_000581F.g46.t1 [mitochondrion] (Humulus lupulus var. lupulus)
Psat4g026320 [mitochondrion] (Pisum sativum)
Jr07_18380 [mitochondrion] (Juglans regia)
LOC114393542 [mitochondrion] (Glycine soja)
GSBRNA2T00028706001 [mitochondrion] (Brassica napus)
LPERR03G33800 [mitochondrion] (Leersia perrieri)
ORGLA03G0364700 [mitochondrion] (Oryza glaberrima)
Tritim_EIv0.3_0699720 [mitochondrion] (Triticum timopheevii subsp. timopheevii)
orange1.1g032695m [mitochondrion] (Citrus sinensis)
GSBRNA2T00028707001 [mitochondrion] (Brassica napus)
gene-LSAT_3X26221 [mitochondrion] (Lactuca sativa)
FRAEX38873_v2_000177780 [mitochondrion] (Fraxinus excelsior (European ash))
Mp5g11250 [mitochondrion] (Marchantia polymorpha)
Csa19g023550 [mitochondrion] (Camelina sativa)
orange1.1g033892m [mitochondrion] (Citrus sinensis)
A4A49_31437 [mitochondrion] (Nicotiana attenuata)
gene-BT93_C0749 [mitochondrion] (Corymbia citriodora)
Homologues of fumarylacetoacetase [mitochondrion] (Arabidopsis lyrata)
Homologues of fumarylacetoacetase [mitochondrion] (Arabidopsis thaliana)
Homologues of fumarylacetoacetase [mitochondrion] (Arachis hypogaea)
Homologues of fumarylacetoacetase [mitochondrion] (Avena sativa)
Homologues of fumarylacetoacetase [mitochondrion] (Beta vulgaris)
Homologues of fumarylacetoacetase [mitochondrion] (Brassica juncea)
Homologues of fumarylacetoacetase [mitochondrion] (Brassica napus)
Homologues of fumarylacetoacetase [mitochondrion] (Brassica oleracea)
Homologues of fumarylacetoacetase [mitochondrion] (Brassica rapa)
Homologues of fumarylacetoacetase [mitochondrion] (Camelina sativa)
Homologues of fumarylacetoacetase [mitochondrion] (Capsella rubella)
Homologues of fumarylacetoacetase [mitochondrion] (Chenopodium quinoa)
Homologues of fumarylacetoacetase [mitochondrion] (Citrus clementina)
Homologues of fumarylacetoacetase [mitochondrion] (Citrus sinensis)
Homologues of fumarylacetoacetase [mitochondrion] (Corymbia citriodora)
Homologues of fumarylacetoacetase [mitochondrion] (Eucalyptus grandis)
Homologues of fumarylacetoacetase [mitochondrion] (Galdieria sulphuraria)
Homologues of fumarylacetoacetase [mitochondrion] (Glycine max)
Homologues of fumarylacetoacetase [mitochondrion] (Glycine soja)
Homologues of fumarylacetoacetase [mitochondrion] (Humulus lupulus)
Homologues of fumarylacetoacetase [mitochondrion] (Humulus lupulus var. lupulus)
Homologues of fumarylacetoacetase [mitochondrion] (Ipomoea triloba)
Homologues of fumarylacetoacetase [mitochondrion] (Lactuca sativa)
Homologues of fumarylacetoacetase [mitochondrion] (Nymphaea colorata)
Homologues of fumarylacetoacetase [mitochondrion] (Oryza australiensis)
Homologues of fumarylacetoacetase [mitochondrion] (Oryza meyeriana var. granulata)
Homologues of fumarylacetoacetase [mitochondrion] (Oryza officinalis)
Homologues of fumarylacetoacetase [mitochondrion] (Pistacia vera)
Homologues of fumarylacetoacetase [mitochondrion] (Quercus lobata)
Homologues of fumarylacetoacetase [mitochondrion] (Quercus suber)
Homologues of fumarylacetoacetase [mitochondrion] (Salvia hispanica)
Homologues of fumarylacetoacetase [mitochondrion] (Selaginella moellendorffii)
Homologues of fumarylacetoacetase [mitochondrion] (Triticum aestivum)
Homologues of fumarylacetoacetase [mitochondrion] (Triticum dicoccoides)
Homologues of fumarylacetoacetase [mitochondrion] (Triticum spelta subsp. spelta)
Homologues of fumarylacetoacetase [mitochondrion] (Triticum timopheevii subsp. timopheevii)
Homologues of fumarylacetoacetase [mitochondrion] (Triticum turgidum)
Homologues of fumarylacetoacetase [mitochondrion] (Zoysia japonica)
NC5G0159490 [mitochondrion] (Nymphaea colorata)
A08p018100.1_BraROA [mitochondrion] (Brassica rapa)
GrLcomp98378_c1_seq1 [mitochondrion] (Oryza meyeriana var. granulata)
OMERI03G36440 [mitochondrion] (Oryza meridionalis)
A01p049520.1_BraROA [mitochondrion] (Brassica rapa)
AeUmb.TA1851.r1.6UG0009180 [mitochondrion] (Aegilops umbellulata)
BGIOSGA013857 [mitochondrion] (Oryza sativa Indica Group)
GSBRNA2T00040134001 [mitochondrion] (Brassica napus)
Bo1g123320 [mitochondrion] (Brassica oleracea)
SECCE4Rv1G0223940 [mitochondrion] (Secale cereale)
CICLE_v10006783mg [mitochondrion] (Citrus clementina)
BAA17682 [mitochondrion] (Synechocystis sp. PCC 6803)
OfLcomp6814_c0_seq1 [mitochondrion] (Oryza officinalis)
Solyc01g066480.3 [mitochondrion] (Solanum lycopersicum)
BjuB07g17750S [mitochondrion] (Brassica juncea)
AVESA.00001b.r3.4Ag0000944 [mitochondrion] (Avena sativa)
EVM0012175 [mitochondrion] (Pistacia vera)
OGLUM03G39520 [mitochondrion] (Oryza glumaepatula)
GrLcomp97955_c0_seq1 [mitochondrion] (Oryza meyeriana var. granulata)
QL06p010208 [mitochondrion] (Quercus lobata)
B456_002G028600 [mitochondrion] (Gossypium raimondii)
GSCOC_T00025773001 [mitochondrion] (Coffea canephora)
Tp57577_TGAC_v2_gene3211 [mitochondrion] (Trifolium pratense)
T459_02352 [mitochondrion] (Capsicum annuum)
gene-LATHSAT_LOCUS17089 [mitochondrion] (Lathyrus sativus)
orange1.1g039682m [mitochondrion] (Citrus sinensis)
DRNTG_23866 [mitochondrion] (Dioscorea rotundata)
NC10G0044160 [mitochondrion] (Nymphaea colorata)
evm.TU.07.410 [mitochondrion] (Cannabis sativa)
HUMLU_pri_000581F.g43.t2 [mitochondrion] (Humulus lupulus var. lupulus)
gene27097 [mitochondrion] (Medicago truncatula)
Vitvi05g00035 [mitochondrion] (Vitis vinifera)
MELO3C006680.2 [mitochondrion] (Cucumis melo)
OBART03G39630 [mitochondrion] (Oryza barthii)
C5167_011959 [mitochondrion] (Papaver somniferum)
SELMODRAFT_439713 [mitochondrion] (Selaginella moellendorffii)
fgenesh2_kg.7__2724__AT4G15940.1 [mitochondrion] (Arabidopsis lyrata)
Potri.010G011000.v4.1 [mitochondrion] (Populus trichocarpa)
TRITD4Bv1G026530 [mitochondrion] (Triticum turgidum)
orange1.1g033723m [mitochondrion] (Citrus sinensis)
TanjilG_29111 [mitochondrion] (Lupinus angustifolius)
JcCB0365551.10 [mitochondrion] (Jatropha curcas)
LOC114420026 [mitochondrion] (Glycine soja)
RchiOBHm_Chr4g0423181 [mitochondrion] (Rosa chinensis)
SIN_1016222 [mitochondrion] (Sesamum indicum)
gene-CFP56_35951 [mitochondrion] (Quercus suber)
BjuB07g44360S [mitochondrion] (Brassica juncea)
Csa10g030010 [mitochondrion] (Camelina sativa)
ONIVA03G41490 [mitochondrion] (Oryza nivara)
TraesCS4A02G236400 [mitochondrion] (Triticum aestivum)
AT4G15940 [mitochondrion] (Arabidopsis thaliana)
Ccrd_002851 [mitochondrion] (Cynara cardunculus var. scolymus)
Carubv10005686m [mitochondrion] (Capsella rubella)
GSBRNA2T00057553001 [mitochondrion] (Brassica napus)
g26676 [mitochondrion] (Salvia hispanica)
Araip.6YJ7D [mitochondrion] (Arachis ipaensis)
EVM0024546 [mitochondrion] (Pistacia vera)
AUR62018079 [mitochondrion] (Chenopodium quinoa)
AVESA.00001b.r3.4Dg0001299 [mitochondrion] (Avena sativa)
HUMLU_hap_000581F_004.g1.t1 [mitochondrion] (Humulus lupulus)
orange1.1g032707m [mitochondrion] (Citrus sinensis)
BRADI_1g14790v3 [mitochondrion] (Brachypodium distachyon)
Cla021756 [mitochondrion] (Citrullus lanatus)
AUR62001364 [mitochondrion] (Chenopodium quinoa)
TraesCS4D02G077400 [mitochondrion] (Triticum aestivum)
gene-BT93_C0720 [mitochondrion] (Corymbia citriodora)
GLYMA_07G245900 [mitochondrion] (Glycine max)
PHAVU_003G106800g [mitochondrion] (Phaseolus vulgaris)
Csa_3G164480 [mitochondrion] (Cucumis sativus)
Bo6g105160 [mitochondrion] (Brassica oleracea)
arahy.Tifrunner.gnm2.ann2.Ah11g427800 [mitochondrion] (Arachis hypogaea)
Carubv10014572m [mitochondrion] (Capsella rubella)
KYUSg_chr4.18488 [mitochondrion] (Lolium perenne)
AuLcomp16347_c0_seq1 [mitochondrion] (Oryza australiensis)
CICLE_v10005665mg [mitochondrion] (Citrus clementina)
Cross References
UniProt