fumarylacetoacetate hydrolase

Stable Identifier
R-OSA-1125883
Type
Protein [EntityWithAccessionedSequence]
Species
Oryza sativa
Synonyms
phys-ent-participant15040
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:Q0DR40 Os03g0421700
Gene Names
Os03g0421700, Os03g0421500, LOC_OS03G30800, OSNPB_030421700
Participant Of
hasMember
Inferred To
AT3G16700 [mitochondrion] (Arabidopsis thaliana)
Vigun03g420700.v1.2 [mitochondrion] (Vigna unguiculata)
g20210 [mitochondrion] (Arabidopsis halleri)
gene-BT93_H0829 [mitochondrion] (Corymbia citriodora)
AMTR_s00183p00050040 [mitochondrion] (Amborella trichopoda)
ORGLA03G0202400 [mitochondrion] (Oryza glaberrima)
TraesTSP4B01G082900 [mitochondrion] (Triticum spelta subsp. spelta)
BGIOSGA012897 [mitochondrion] (Oryza sativa Indica Group)
GSBRNA2T00022769001 [mitochondrion] (Brassica napus)
GLYMA_17G028100 [mitochondrion] (Glycine max)
BVRB_3g067140 [mitochondrion] (Beta vulgaris)
CBR_g26355 [mitochondrion] (Chara braunii)
QL06p010180 [mitochondrion] (Quercus lobata)
TCM_021244 [mitochondrion] (Theobroma cacao)
Csa12g053610 [mitochondrion] (Camelina sativa)
OMERI03G19660 [mitochondrion] (Oryza meridionalis)
TraesTSP4D01G081000 [mitochondrion] (Triticum spelta subsp. spelta)
HanXRQr2_Chr06g0251801 [mitochondrion] (Helianthus annuus)
Homologues of fumarylacetoacetate hydrolase [mitochondrion] (Brassica napus)
Homologues of fumarylacetoacetate hydrolase [mitochondrion] (Brassica rapa)
Homologues of fumarylacetoacetate hydrolase [mitochondrion] (Citrus clementina)
OBART03G21770 [mitochondrion] (Oryza barthii)
Manes.03G198200.v8.1 [mitochondrion] (Manihot esculenta)
Csa15g021590 [mitochondrion] (Camelina sativa)
Tritim_EIv0.3_0838510 [mitochondrion] (Triticum timopheevii subsp. timopheevii)
EUGRSUZ_C01303 [mitochondrion] (Eucalyptus grandis)
Vfaba.Hedin2.R1.4g194200 [mitochondrion] (Vicia faba var. faba)
Bo8g042180 [mitochondrion] (Brassica oleracea)
gene-LSAT_3X24101 [mitochondrion] (Lactuca sativa)
gene-BT93_C0755 [mitochondrion] (Corymbia citriodora)
PRUPE_1G125100 [mitochondrion] (Prunus persica)
FCD_00023334 [mitochondrion] (Ficus carica)
Csa11g034280 [mitochondrion] (Camelina sativa)
Vradi07g25080 [mitochondrion] (Vigna radiata)
OPUNC03G20520 [mitochondrion] (Oryza punctata)
itb13g22130 [mitochondrion] (Ipomoea triloba)
arahy.Tifrunner.gnm2.ann2.Ah01g358900 [mitochondrion] (Arachis hypogaea)
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)
OE9A046022 [mitochondrion] (Olea europaea)
OE9A046022 [mitochondrion] (Olea europaea var. sylvestris)
Csa01g019380 [mitochondrion] (Camelina sativa)
C.cajan_04306 [mitochondrion] (Cajanus cajan)
Psat4g026320 [mitochondrion] (Pisum sativum)
Jr07_18380 [mitochondrion] (Juglans regia)
LOC114393542 [mitochondrion] (Glycine soja)
LPERR03G33800 [mitochondrion] (Leersia perrieri)
Tritim_EIv0.3_0699720 [mitochondrion] (Triticum timopheevii subsp. timopheevii)
GSBRNA2T00028707001 [mitochondrion] (Brassica napus)
ORUFI03G22460 [mitochondrion] (Oryza rufipogon)
FRAEX38873_v2_000177780 [mitochondrion] (Fraxinus excelsior (European ash))
ONIVA03G23440 [mitochondrion] (Oryza nivara)
Mp5g11250 [mitochondrion] (Marchantia polymorpha)
Csa19g023550 [mitochondrion] (Camelina sativa)
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] (Brassica juncea)
Homologues of fumarylacetoacetase [mitochondrion] (Brassica oleracea)
Homologues of fumarylacetoacetase [mitochondrion] (Camelina sativa)
Homologues of fumarylacetoacetase [mitochondrion] (Corymbia citriodora)
Homologues of fumarylacetoacetase [mitochondrion] (Glycine max)
Homologues of fumarylacetoacetase [mitochondrion] (Glycine soja)
Homologues of fumarylacetoacetase [mitochondrion] (Pistacia vera)
Homologues of fumarylacetoacetase [mitochondrion] (Triticum spelta subsp. spelta)
Homologues of fumarylacetoacetase [mitochondrion] (Triticum timopheevii subsp. timopheevii)
A08p018100.1_BraROA [mitochondrion] (Brassica rapa)
A01p049520.1_BraROA [mitochondrion] (Brassica rapa)
AeUmb.TA1851.r1.6UG0009180 [mitochondrion] (Aegilops umbellulata)
GSBRNA2T00040134001 [mitochondrion] (Brassica napus)
Bo1g123320 [mitochondrion] (Brassica oleracea)
Solyc01g066480.3 [mitochondrion] (Solanum lycopersicum)
BjuB07g17750S [mitochondrion] (Brassica juncea)
AVESA.00001b.r3.4Ag0000944 [mitochondrion] (Avena sativa)
EVM0012175 [mitochondrion] (Pistacia vera)
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)
NC10G0044160 [mitochondrion] (Nymphaea colorata)
evm.TU.07.410 [mitochondrion] (Cannabis sativa)
gene27097 [mitochondrion] (Medicago truncatula)
Vitvi05g00035 [mitochondrion] (Vitis vinifera)
MELO3C006680.2 [mitochondrion] (Cucumis melo)
C5167_011959 [mitochondrion] (Papaver somniferum)
fgenesh2_kg.7__2724__AT4G15940.1 [mitochondrion] (Arabidopsis lyrata)
TanjilG_29111 [mitochondrion] (Lupinus angustifolius)
LOC114420026 [mitochondrion] (Glycine soja)
RchiOBHm_Chr4g0423181 [mitochondrion] (Rosa chinensis)
SIN_1016222 [mitochondrion] (Sesamum indicum)
BjuB07g44360S [mitochondrion] (Brassica juncea)
Csa10g030010 [mitochondrion] (Camelina sativa)
AT4G15940 [mitochondrion] (Arabidopsis thaliana)
OGLUM03G22580 [mitochondrion] (Oryza glumaepatula)
Ccrd_002851 [mitochondrion] (Cynara cardunculus var. scolymus)
GSBRNA2T00057553001 [mitochondrion] (Brassica napus)
EVM0024546 [mitochondrion] (Pistacia vera)
AUR62018079 [mitochondrion] (Chenopodium quinoa)
AVESA.00001b.r3.4Dg0001299 [mitochondrion] (Avena sativa)
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)
CICLE_v10005665mg [mitochondrion] (Citrus clementina)
Cross References
UniProt