3.7.1.2: fumarylacetoacetase

This is an abbreviated version!
For detailed information about fumarylacetoacetase, go to the full flat file.

Word Map on EC 3.7.1.2

3.7.1.2

tyrosinemia

hereditary

flavone

hepatocytes

anticipatory

aviation

succinylacetone

schedule

oscillator

rhythm

circadian

entrain

frontal

ntbc

flight

meal

asymmetry

clock

federal

aircraft

locomotor

fanconi

repopulation

food-entrainable

suprachiasmatic

civil

taurine

n-2-fluorenylacetamide

accident

certification

antitumour

medicine

homogentisate

4-hydroxyphenylpyruvate

mealtime

maleylacetoacetate

rickets

aerospace

dorsomedial

xaa

electroencephalography

hepatorenal

civilian

zeitgeber

4-aminoantipyrine

anteversion

dipyrone

light-entrainable

biotechnology

degradation

Reaction

Synonyms

4-fumarylacetoacetate fumarylhydrolase, acylpyruvase, AtFAH, beta-diketonase, diketo acid hydrolase, EaFAH, FAA, FAA hydrolase, FAH, fumarylacetoacetase, fumarylacetoacetate hydrolase, HmgB, mFAH, NagK, SSCD1, TTHA0809

ECTree

3 Hydrolases

3.7 Acting on carbon-carbon bonds

3.7.1 In ketonic substances

3.7.1.2 fumarylacetoacetase

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Results	in table
13789	AA Sequence
30	Application
1	CAS Registry Number
16	Cloned(Commentary)
5	Crystallization (Commentary)
1273	Disease/ Diagnostics
2	Expression
15	General Information
39	Inhibitors
3	Ki Value [mM]
15	KM Value [mM]
4	Localization
11	Metals/Ions
17	Molecular Weight [Da]
22	Natural Substrates/ Products (Substrates)
1	Organic Solvent Stability
57	Organism
7	Pathway
18	PDB ID
11	pH Optimum
3	pH Range
1	pI Value
35	Protein Variants
20	Purification (Commentary)
3	Reaction
7	Reaction Type
57	Reference
43	Source Tissue
34	Specific Activity [micromol/min/mg]
4	Storage Stability
84	Substrates and Products (Substrate)
15	Subunits
75	Synonyms
1	Systematic Name
3	Temperature Optimum [°C]
1	Temperature Stability [°C]
1	Turnover Number [1/s]

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Reference on EC 3.7.1.2 - fumarylacetoacetase

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289238

Meister, A.; Greenstein, J.P.

Enzymatic hydrolysis of 2,4-diketo acids

J. Biol. Chem.

175

573-588

1948

Oryctolagus cuniculus, Felis catus, Mus musculus, Rattus norvegicus

18880754

289239

Connors, W.M.; Stotz, E.

The purification and properties of a triacetic acid-hydrolyzing enzyme

J. Biol. Chem.

178

881-890

1949

Bos taurus

18117010

289240

Edwards, S.W.; Knox, W.E.

Homogentisate metabolism: The isomerization of meleylacetoacetate by an enzyme which requires glutathione

J. Biol. Chem.

220

79-91

1956

Bos taurus, Rattus norvegicus

13319328

289241

Hsiang,.H.; Sim, S.S.; Mahuran, D.J.; Schmidt, Jr., D.E.

Purification and properties of a diketo acid hydrolase from beef liver

Biochemistry

2098-2102

1972

Bos taurus

5027618

289242

Braun, C.V.; Schmidt, Jr., D.E.

Inhibition of fumarylacetoacetate fumarylhydrolase by monovalent anions

Biochemistry

4878-4881

1973

Bos taurus

4357550

289243

Mahuran, D.J.; Angus, R.H.; Braun, C.V.; Sim, S.S.; Schmidt, Jr., D.E.

Characterization and substrate specificity of fumarylacetoacetate fumarylhydrolase

Can. J. Biochem.

1-8

1977

Bos taurus

13916

289244

Lindblad, B.; Lindstedt, S.; Steen, G.

On the enzymic defects in hereditary tyrosinemia

Proc. Natl. Acad. Sci. USA

4641-4645

1977

Homo sapiens

270706

289245

Doshi, K.S.; Schmidt, Jr., D.E.

Localization of fumarylacetoacetate fumarylhydrolase in rat liver

Can. J. Biochem.

866-868

1977

Rattus norvegicus

728842

289246

Nagainis, M.P.; Pu, W.; Cheng, B.; Taylor, K.E.; Schmidt, Jr., D.E.

Effects of pH and sulfhydryl specific reagents on 4-fumarylacetoacetate fumarylhydrolase

Biochim. Biophys. Acta

657

203-211

1981

Bos taurus

7213744

289247

Van Faassen, H.; van den Berg, I.E.T.; Berger, R.

Purification of human liver fumarylacetoacetase using immunoaffinity chromatography

J. Biochem. Biophys. Methods

317-324

1990

Homo sapiens

2195095

289248

Laberge, C.; Grenier, A.; Valet, J.P.; Morissette, J.

Fumarylacetoacetase measurement as a mass-screening procedure for hereditary tyrosinemia type I

Am. J. Hum. Genet.

325-328

1990

Homo sapiens

2378358

289249

Phaneuf, D.; Labelle, Y.; Berube, D.; Arden, K.; Cavenee, W.; Gagne, R; Tanguay, R.M.

Cloning and expression of the cDNA encoding human fumarylacetoacetate hydrolase, the enzyme deficient in hereditary tyrosinemia: Assignment of the gene to chromosome 15

Am. J. Hum. Genet.

525-535

1991

Homo sapiens

1998338

289250

Labelle, Y.; Phaneuf, D.; Tanguay, R.M.

Cloning and expression analysis of a cDNA encoding fumarylacetoacetate hydrolase: post-transcriptional modulation in rat liver and kidney

Gene

104

197-202

1991

Rattus norvegicus

1916290

289251

Klebig, M.L.; Russell, L.B.; Rinchik, E.M.

Murine fumarylacetoacetate hydrolase (Fah) gene is disrupted by a neonatally lethal albino deletion that defines the hepatocyte-specific developmental regulation 1 (hsdr-1) locus

Proc. Natl. Acad. Sci. USA

1363-1367

1992

Mus musculus

1741389

289252

Kvittingen, E.A.; Rootwelt, H.; Brandtzaeg, P.; Bergan, A.; Berger, R.

Hereditary tyrosinemia type I

J. Clin. Invest.

1816-1821

1993

Homo sapiens

8473520

289253

Labelle, Y.; Puymirat, J.; Tanguay, R.M.

Localization of cells in the rat brain expressing fumarylacetoacetate hydrolase, the deficient enzyme in hereditary tyrosinemia type 1

Biochim. Biophys. Acta

1180

250-256

1993

Homo sapiens, Rattus norvegicus

8422430

289254

Grompe, M.; Al-Dhalimy, M.; Finegold, M.; Ou, C.N.; Burlingame, T.; Kennaway, N.G.; Soriano, P.

Loss of fumarylacetoacetate hydrolysis is responsible for the neonatal hepatic dysfunction phenotype of lethal albino mice

Genes Dev.

2298-2307

1993

Mus musculus

8253378

289255

Labelle, Y.; Phaneuf, D.; Leclerc, B.; Tanguay, R.M.

Characterization of the human fumarylacetoacetate hydrolase gene and identification of a missense mutation abolishing enzymatic activity

Hum. Mol. Genet.

941-946

1993

Homo sapiens

8364576

289256

Awata, H.; Endo, F.; Tanoue, A.; Kitano, A.; Nakano, Y.; Matsuda, I.

Structural organization and analysis of the human fumarylacetoacetate hydrolase gene in tyrosinemia type I

Biochim. Biophys. Acta

1226

168-172

1994

Homo sapiens

8204664

289257

Timm, D.E.; Mueller, H.A.; Bhanumoorthy, P.; Harp, J.M.; Bunick, G.J.

Crystal structure and mechanism of a carbon-carbon bond hydrolase

Structure

1023-1033

1999

Mus musculus

10508789

655997

Bateman, R.L.; Bhanumoorthy, P.; Witte, J.F.; McClard, R.W.; Grompe, M.; Timm, D.E.

Mechanistic inferences from the crystal structure of fumarylacetoacetate hydrolase with a bound phosphorus-based inhibitor

J. Biol. Chem.

276

15284-15291

2001

Homo sapiens, Mus musculus

11154690

657866

Grogan, G.

Emergent mechanistic diversity of enzyme-catalysed beta-diketone cleavage

Biochem. J.

388

721-730

2005

Bos taurus, Mus musculus

15934927

669086

Arias-Barrau, E.; Olivera, E.R.; Luengo, J.M.; Fernandez, C.; Galan, B.; Garcia, J.L.; Diaz, E.; Minambres, B.

The homogentisate pathway: a central catabolic pathway involved in the degradation of L-phenylalanine, L-tyrosine, and 3-hydroxyphenylacetate in Pseudomonas putida

J. Bacteriol.

186

5062-5077

2004

Pseudomonas putida, Pseudomonas putida U-dhmgB

15262943

670622

Dixon, D.P.; Edwards, R.

Enzymes of tyrosine catabolism in Arabidopsis thaliana

Plant Sci.

171

360-366

2006

Arabidopsis thaliana

22980205

670957

Williams, R.E.; Lock, E.A.

D-serine-induced nephrotoxicity: possible interaction with tyrosine metabolism

Toxicology

201

231-238

2004

Rattus norvegicus

15297036

684183

Mizutani, H.; Kunishima, N.

Purification, crystallization and preliminary X-ray analysis of the fumarylacetoacetase family member TTHA0809 from Thermus thermophilus HB8

Acta Crystallogr. Sect. F

792-794

2007

Thermus thermophilus

17768357

684964

Bateman, R.L.; Ashworth, J.; Witte, J.F.; Baker, L.J.; Bhanumoorthy, P.; Timm, D.E.; Hurley, T.D.; Grompe, M.; McClard, R.W.

Slow-onset inhibition of fumarylacetoacetate hydrolase by phosphinate mimics of the tetrahedral intermediate: kinetics, crystal structure and pharmacokinetics

Biochem. J.

402

251-260

2007

Homo sapiens, Mus musculus (P35505), Mus musculus

17064256

686266

Kang, B.R.; Han, S.H.; Cho, S.M.; Anderson, A.J.; Kim, I.S.; Park, S.K.; Kim, Y.C.

Characterization of a homogentisate dioxygenase mutant in Pseudomonas chlororaphis O6

Curr. Microbiol.

145-149

2008

Pseudomonas chlororaphis, Pseudomonas chlororaphis O6

18074175

687809

Fisher, A.L.; Page, K.E.; Lithgow, G.J.; Nash, L.

The Caenorhabditis elegans K10C2.4 gene encodes a member of the fumarylacetoacetate hydrolase family: a Caenorhabditis elegans model of type I tyrosinemia

J. Biol. Chem.

283

9127-9135

2008

Caenorhabditis elegans (Q94272), Caenorhabditis elegans

18227072

697797

Eggenhofer, E.; Popp, F.C.; Renner, P.; Slowik, P.; Neuwinger, A.; Piso, P.; Geissler, E.K.; Schlitt, H.J.; Dahlke, M.H.

Allogeneic bone marrow transplantation restores liver function in Fah-knockout mice

Exp. Hematol.

1507-1513

2008

Mus musculus (P35505)

18715687

699824

Werner, T.V.; Hoermannsperger, G.; Schuemann, K.; Hoelzlwimmer, G.; Tsuji, S.; Haller, D.

Intestinal epithelial cell proteome from wild type and TNFARE/WT mice: effect of iron on the development of chronic ileitis

J. Proteome Res.

3252-3264

2009

Mus musculus

19422269

706809

Battilana, J.; Costantini, L.; Emanuelli, F.; Sevini, F.; Segala, C.; Moser, S.; Velasco, R.; Versini, G.; Stella Grando, M.

The 1-deoxy-D: -xylulose 5-phosphate synthase gene co-localizes with a major QTL affecting monoterpene content in grapevine

Theor. Appl. Genet.

118

653-669

2009

Vitis vinifera x Vitis vinifera, Vitis vinifera x Vitis riparia

19037624

734103

Liu, Y.; Xia, W.; Yang, P.; Zhang, S.; Shi, Z.; Tang, H.; Zhang, L.

Cloning and expression of fumarylacetoacetate hydrolase derived from marine yeast Rhodosporidium diobovatum

J. Basic Microbiol.

1082-1093

2015

Rhodotorula diobovata (A0A076VF18), Rhodotorula diobovata, Rhodotorula diobovata MCCC 2A00023 (A0A076VF18)

25832819

734967

Han, C.; Ren, C.; Zhi, T.; Zhou, Z.; Liu, Y.; Chen, F.; Peng, W.; Xie, D.

Disruption of fumarylacetoacetate hydrolase causes spontaneous cell death under short-day conditions in Arabidopsis

Plant Physiol.

162

1956-1964

2013

Arabidopsis thaliana (Q8RW90)

23743712

755976

Yoo, W.; Lee, C.W.; Kim, B.Y.; Huong Luu Le, L.T.; Park, S.H.; Kim, H.W.; Shin, S.C.; Kim, K.K.; Lee, J.H.; Kim, T.D.

Structural and functional analysis of a dimeric fumarylacetoacetate hydrolase (EaFAH) from psychrophilic Exiguobacterium antarcticum

Biochem. Biophys. Res. Commun.

509

773-778

2019

Exiguobacterium antarcticum (K0A9N9), Exiguobacterium antarcticum, Exiguobacterium antarcticum B7 (K0A9N9)

30630595

756026

Weiss, A.K.H.; Loeffler, J.R.; Liedl, K.R.; Gstach, H.; Jansen-Duerr, P.

The fumarylacetoacetate hydrolase (FAH) superfamily of enzymes multifunctional enzymes from microbes to mitochondria

Biochem. Soc. Trans.

295-309

2018

Acinetobacter baumannii (A0A0D5YDA5), Cupriavidus gilardii (A0A0M3SVN7), Phaeobacter gallaeciensis (A0A1B0ZSC0), Homo sapiens (P16930), Homo sapiens, Cupriavidus gilardii CR3 (A0A0M3SVN7)

29487229

757221

Macias, I.; Lain, A.; Bernardo-Seisdedos, G.; Gil, D.; Gonzalez, E.; Falcon-Perez, J.M.; Millet, O.

Hereditary tyrosinemia type I-associated mutations in fumarylacetoacetate hydrolase reduce the enzyme stability and increase its aggregation rate

J. Biol. Chem.

294

13051-13060

2019

Homo sapiens (P16930)

31300554

758089

Zhi, T.; Zhou, Z.; Huang, Y.; Han, C.; Liu, Y.; Zhu, Q.; Ren, C.

Sugar suppresses cell death caused by disruption of fumarylacetoacetate hydrolase in Arabidopsis

Planta

244

557-571

2016

Arabidopsis thaliana (Q8RW90)

27097641

758094

Huang, L.; Hu, C.; Cai, W.; Zhu, Q.; Gao, B.; Zhang, X.; Ren, C.

Fumarylacetoacetate hydrolase is involved in salt stress response in Arabidopsis

Planta

248

499-511

2018

Arabidopsis thaliana (Q8RW90), Arabidopsis thaliana Col-0 (Q8RW90)

29785518