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1 quence that spans four exons of the fumarate hydratase.
2 ially larger degree of covalency for nitrile hydratase.
3 y redox behavior at the iron site in nitrile hydratase.
4 rtant to the catalytic activity of enoyl-CoA hydratase.
5 ntaining protein and mitochondrial aconitate hydratase.
6  required for the high kcat of the enoyl-CoA hydratase.
7 tified as the catalytic residue of enoyl-CoA hydratase.
8 , of the Mycobacterium tuberculosis fumarate hydratase.
9 te determination of the activity of fumarate hydratase.
10 and RpfF shows some relatedness to enoyl CoA hydratases.
11 gy, respectively, to other sequenced nitrile hydratases.
12  that exceeding nutrients suppress Enoyl-CoA hydratase-1 (ECHS1) activity by inducing its acetylation
13             Herein, we report that enoyl-CoA hydratase-1 (ECHS1), the enzyme involved in the oxidatio
14                    Aconitase-2 and enoyl-CoA-hydratase-1 expression levels were decreased in L-NMMA-t
15       Depletion of aconitase-2 and enoyl-CoA-hydratase-1 resulted in the inhibition of the Krebs cycl
16  aldehyde dehydrogenase-1; D204 in enoyl CoA hydratase-1), as well as residues of unknown function (e
17  depletion of both aconitase-2 and enoyl-CoA-hydratase-1.
18 ntribute to renal function include enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase (EHHADH) and a
19 es: fatty acyl-CoA oxidase (ACOX), enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase (HD), and thio
20 l-CoA thiolase (THIO), peroxisomal enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase (HD), peroxiso
21 noate hydrolase, 2-hydroxypenta-2,4-dienoate hydratase, 4-hydroxy-2-oxovalerate aldolase, and acetald
22                                     Fumarate hydratase, a component of the tricarboxylic acid cycle,
23  the iron(III) site of Fe-containing nitrile hydratase, a designed ligand PyPSH(4) with two carboxami
24 2 (formerly Brevibacterium sp. R312) nitrile hydratase, a novel non-heme iron enzyme, have a large nu
25 tion of the nitrile substrate to the nitrile hydratase active site low spin Fe(3+) center.
26 ng with mechanisms for the decarboxylase and hydratase activities.
27           The results provide evidence for a hydratase activity and set the stage to use the 3-halopr
28 on to the decarboxylase activity, MSAD has a hydratase activity as demonstrated by the MSAD-catalyzed
29 E111Q4-OD/VPH complexes, which retained full hydratase activity but had little decarboxylase activity
30 led to a 28-fold reduction in 2-butenoyl-CoA hydratase activity in a preparation of organelles.
31  results provide additional evidence for the hydratase activity of MSAD and further support for the h
32  and Arg-75 have also been implicated in the hydratase activity of MSAD in which 2-oxo-3-pentynoate i
33 a mechanism-based inhibitor activated by the hydratase activity of MSAD, have been determined.
34 singly, ECH exhibiting less than 2% residual hydratase activity retains essentially 100% beta-elimina
35 nd in Bacillus subtilis, exhibit a low level hydratase activity that converts trans-3-haloacrylates t
36                             A weak enoyl-CoA hydratase activity was detected for both DpgB and DpgD.
37 sing" homologue of Glu144 fails to introduce hydratase activity with the substrate analogues.
38 A dehydrogenase also has intrinsic enoyl-CoA hydratase activity, a property of other members of the a
39 exhibit beta-methylcrotonyl-coenzyme A (CoA) hydratase activity, as we predicted.
40 H-CoA effects rapid and irreversible loss of hydratase activity.
41 ype complex, but is devoid of any detectable hydratase activity.
42 y with a trans-o-hydroxybenzylidene-pyruvate hydratase-aldolase gene (pbhC).
43  from pharmacological inhibition of fumarate hydratase (also known as fumarase).
44 transcripts, that this gene encodes fumarate hydratase, an enzyme of the tricarboxylic acid cycle.
45  been obtained for HD-CoA bound to enoyl-CoA hydratase, an enzyme system that has also previously bee
46                                     Fumarate hydratase, an essential enzyme in the tricarboxylic acid
47 ultifunctional enzyme that minimally encodes hydratase and dehydrogenase activities.
48 their sequence, OdaA and OdaI have predicted hydratase and dioxygenase reductase activities, respecti
49  another stable complex, PaaFG, an enoyl-CoA hydratase and enoyl-Coa isomerase, both belonging to the
50    These results imply that loss of fumarate hydratase and fumarate accumulation contribute to the ag
51 l-CoA (HD-CoA), bound to wild-type enoyl-CoA hydratase and G141P, a mutant in which a hydrogen bond t
52 en bound as an enolate to MCAD and enoyl-CoA hydratase and is used to rationalize the observation tha
53 e reactions, two of which (2-trans enoyl-CoA hydratase and L-3-hydroxyacyl-CoA dehydrogenase) are cor
54  the active site of long-chain 2,3-enoyl-CoA hydratase and long-chain 3-ketoacyl-CoA thiolase.
55 DNA-encoded mitochondrial genes for fumarate hydratase and succinate dehydrogenase have been linked t
56 des a linker domain between the NH2-terminal hydratase and the COOH-terminal 3-hydroxyacyl-CoA dehydr
57           Here we show that loss of fumarate hydratase and the subsequent accumulation of fumarate in
58 erties of 3-ketoacyl-CoA thiolase, enoyl-CoA hydratase, and delta 3-cis-delta 2-trans-enoyl-CoA isome
59 l carcinoma cells with mutations in fumarate hydratase, and in cells with normal mitochondria subject
60 as NADH peroxidase and NADH oxidase, nitrile hydratase, and the hORF6 and AhpC peroxiredoxins.
61 the metal ion in the iron-containing nitrile hydratases are conserved in this enzyme, suggesting that
62                                      Nitrile hydratases are enzymes involved in the conversion of nit
63 finity labeling strategy identified fumarate hydratase as the principal pharmacological target.
64  a significant change in the Km value of the hydratase as well as a 5.9- and 62-fold increase, respec
65 f the mechanism of action of other acetylene hydratases, as well as in the design of antiinfectives t
66 ichia coli enzyme 2-hydroxypentadienoic acid hydratase assembles to form a 20-nm-diameter particle co
67 yl group by 3-vinyl bacteriochlorophyllide a hydratase (BchF) followed by 3-hydroxyethyl bacteriochlo
68 phosphoenolpyruvate carboxykinase, aconitate hydratase, branched-chain alpha-keto acid dehydrogenase
69                         A divergent fumarate hydratase C (fumC) gene lies further upstream.
70  protic residues is directly involved in the hydratase catalysis, the multienzyme complexes with eith
71                                    Enoyl-CoA hydratase catalyzes the hydration of trans-2-crotonyl-Co
72 the tricarboxylic acid cycle enzyme fumarate hydratase cause hereditary leiomyomatosis and renal cell
73          Significantly, binding to enoyl-CoA hydratase causes the chemical shifts of the C1 and C3 HD
74 d a plant resolve in the prokaryotic nitrile hydratase clade.
75 the active site of cobalt containing nitrile hydratase (Co NHase) was prepared.
76  of the crotonase family including enoyl-CoA hydratase (crotonase) and methylmalonyl-CoA decarboxylas
77                     Members of the enoyl-CoA hydratase (crotonase) superfamily catalyze different ove
78 r genes encoded homologs of a 1,2-carotenoid hydratase (CrtC), an O-methyltransferase (CrtF), and two
79 e desaturase (crtU/CT0323), carotenoid 1',2'-hydratase (crtC/CT0301), and carotenoid cis-trans isomer
80                  Many children with fumarate hydratase deficiency do not survive infancy or childhood
81 ittent porphyria; delta amino-levulinic acid hydratase deficiency porphyria; hereditary coproporphyri
82 so occur in the recessive condition fumarate hydratase deficiency, and some parents of people with th
83 cule metabolite that accumulates in fumarate hydratase-deficient cells, plays a key role in cell tran
84                                     Fumarate hydratase-deficient renal cancers are highly aggressive
85 ibute to the aggressive features of fumarate hydratase-deficient tumours.
86 tified a petunia gene encoding cinnamoyl-CoA hydratase-dehydrogenase (PhCHD), a bifunctional peroxiso
87 SD IV contains' a region homologous to yeast hydratase-dehydrogenase-epimerases and to sterol carrier
88 letion analyses confirmed that the enoyl-CoA hydratase/dehydrogenase Fox2p, the putative 3-hydroxypro
89 es show that kidney cancers lacking fumarate hydratase display increased sensitivity to agents that i
90             Isocyanide (formerly isonitrile) hydratase (EC 4.2.1.103) is an enzyme of the DJ-1 superf
91  deficiency of the enzyme fumarase (fumarate hydratase, EC 4.2.1.2) which result in autosomal recessi
92 he crystal structure of the enzyme enoyl-CoA hydratase (ECH) from rat liver with the bound substrate
93 opyl)glycine, against bovine liver enoyl-CoA hydratase (ECH) were characterized.
94 an be directly bioactivated by the enoyl-CoA hydratase (ECH) with the release of 1,2-dichloro-3,3,3-t
95 D 3q1t) has been reported to be an enoyl-CoA hydratase (ECH), but SALSA analysis shows a poor match b
96 rected inactivator of bovine liver enoyl-CoA hydratase (ECH).
97 mologous to mammalian enoyl-coenzyme A (CoA) hydratases, EchA6 is non-catalytic yet essential and bin
98 the gene encoding the 2-methyl-cis-aconitate hydratase enzyme is encoded outside the prpBCDE operon.
99 coding the tricarboxylic acid cycle fumarate hydratase enzyme.
100 ferentially conserved between hydrolases and hydratases established that this position is relevant to
101  of the active site of Fe-containing nitrile hydratase (Fe-NHase), a model complex of the NO-bound ac
102 has extensive homology to the human fumarate hydratase (FH) and encodes a 288-amino acid protein (Mw
103 enes succinate dehydrogenase (SDH), fumarate hydratase (FH) and isocitrate dehydrogenase (IDH), advan
104 uclear-encoded Krebs cycle enzymes, fumarate hydratase (FH) and succinate dehydrogenase (SDHB, -C and
105 icarboxylic acid (TCA) cycle enzyme fumarate hydratase (FH) are associated with a highly malignant fo
106   Succinate dehydrogenase (SDH) and fumarate hydratase (FH) are components of the tricarboxylic acid
107 t succinate dehydrogenase (SDH) and fumarate hydratase (FH) are tumour suppressors and which associat
108                         Mutation of fumarate hydratase (FH) at 1q43 is known to cause the Mendelian s
109 icarboxylic acid (TCA) cycle enzyme fumarate hydratase (FH) cause a hereditary cancer syndrome known
110 uccinate dehydrogenase A (SDHA) and fumarate hydratase (FH) expression.
111 cation of germline mutations in the fumarate hydratase (FH) gene in European families supports it as
112 The tricarboxylic acid cycle enzyme fumarate hydratase (FH) has been identified as a tumor suppressor
113 the tricarboxylic acid cycle enzyme fumarate hydratase (FH) have been linked to an aggressive variant
114 intracellular fumarate, a result of fumarate hydratase (FH) inactivation, but it is not clear how NRF
115                                     Fumarate hydratase (FH) is an enzyme of the tricarboxylic acid cy
116              Patients with germline fumarate hydratase (FH) mutation are predisposed to develop aggre
117 ene encoding the Krebs cycle enzyme fumarate hydratase (FH) predispose to hereditary leiomyomatosis a
118  succinate dehydrogenase (SDH), and fumarate hydratase (FH) that produce oncometabolites that competi
119 utations, biallelic inactivation of fumarate hydratase (FH), and collagen, type IV, alpha 5 and colla
120                       Expression of fumarate hydratase (FH), which regulates urine fumarate accumulat
121 l and mutation of the gene encoding fumarate hydratase (FH).
122 the tricarboxylic acid cycle enzyme fumarate hydratase (FH).
123           Germline mutations of the fumarate hydratase (FH, fumarase) gene are found in the recessive
124  study, we investigated the role of fumarate hydratase (Fh1), a key component of the mitochondrial tr
125                                     Fumarate hydratases (FHs) are essential metabolic enzymes grouped
126    Monofunctional 2-hydroxypentadienoic acid hydratase from Escherichia coli has been purified 3800-f
127 r crystallization of two proteins, enoyl-CoA hydratase from Mycobacterium tuberculosis and dihydrofol
128 r crystallization of two proteins, enoyl-CoA hydratase from Mycobacterium tuberculosis and dihydrofol
129 cond and third shells of the Co-type nitrile hydratase from Pseudomonas putida (ppNHase) that may be
130                                      Nitrile hydratase from Pseudomonas putida NRRL-18668 has been pu
131 data were obtained for the iron-type nitrile hydratase from Rhodococcus equi TG328-2 (ReNHase) using
132        The alphabeta dimer of active nitrile hydratase from Rhodococcus sp. R312 contains one low-spi
133                       The absence of nitrile hydratase from several sequenced species indicates that
134     The fungal-specific enzyme homoaconitate hydratase from this pathway is moderately similar to the
135 requires a 4-carboxy-2-hydroxymuconate (CHM) hydratase (GalB), which has a 12% sequence identity to a
136           Germline mutations in the fumarate hydratase gene (FH) predispose to multiple cutaneous and
137                                  The nitrile hydratase gene in M. brevicollis was believed to have ar
138 nce presented here demonstrates that nitrile hydratase genes are present in multiple eukaryotic super
139 NGS: Here we report the detection of nitrile hydratase genes in five eukaryotic supergroups: opisthok
140 ATP-synthase delta chain and Enoyl-CoenzymeA hydratase, glutathione-s-transferase omega, alpha-1-acid
141 yme A (HD-CoA) bound to the enzyme enoyl-CoA hydratase has been determined using transferred nuclear
142 64 and E144) in the active site of enoyl-CoA hydratase has been probed by site-directed mutagenesis.
143 9 and 9.2, whereas the Glu119 --> Gln mutant hydratase has only a single pKa of 9.5.
144  they are widespread in prokaryotes, nitrile hydratases have only been reported in two eukaryotes: th
145  of the PPARalpha target genes rat enoyl-CoA hydratase (HD) and peroxisomal fatty acyl-CoA oxidase (A
146 derstand the structural basis for isocyanide hydratase (ICH) catalysis, we determined the crystal str
147 2 (formerly Brevibacterium sp. R312) nitrile hydratase in frozen solutions at pH 7 and 9 has been ana
148                      The presence of nitrile hydratases in many other eukaryotic groups is unresolved
149 s recover a clade of eukaryotic-type nitrile hydratases in the Opisthokonta, Amoebozoa, SAR and CCTH;
150  family of nonheme iron enzymes, the nitrile hydratases, in which post-translational oxidation of two
151                    NO-sensitive [4Fe-4S] (de)hydratases, including the Krebs cycle aconitase and the
152            These data suggest that enoyl-CoA hydratase is an important enzyme in the bioactivation of
153                             Loss of fumarate hydratase is associated with suppression of miR-200 and
154                   2-Hydroxypentadienoic acid hydratase is found on many bacterial catabolic pathways
155                         Activity of fumarate hydratase is reduced in lymphoblastoid cells from indivi
156 showed significant homology to the enoyl-CoA hydratase/isomerase enzyme family.
157 sociated with the members of the 2-enoyl-CoA hydratase/isomerase enzyme superfamily are compared to s
158 active site of one member of the 2-enoyl-CoA hydratase/isomerase family, 4-chlorobenzoyl-CoA dehaloge
159 moderate sequence identity to members of the hydratase/isomerase superfamily of enzymes.
160 arity to FabA, but rather is a member of the hydratase/isomerase superfamily.
161 ication of a putative enoyl-coenzyme A (CoA) hydratase/isomerase that is required for synthesis of th
162 se family and that IBR10 resembles enoyl-CoA hydratases/isomerases.
163 at the level of the second enzyme, enoyl-CoA hydratase/L-3-hydroxyacyl-CoA dehydrogenase (L-PBE) of t
164     In this study, the function of enoyl-CoA hydratase/L-3-hydroxyacyl-CoA dehydrogenase (L-PBE), the
165 d transcription from a peroxisomal enoyl-CoA hydratase/l-3-hydroxyacyl-CoA dehydrogenase bifunctional
166 y of CBP and TRAP150, to the mouse enoyl-CoA hydratase/l-3-hydroxyacyl-CoA dehydrogenase gene promote
167 strates bind to the active site of enoyl-CoA hydratase, large spectral changes can be observed.
168 ence identity to a previously identified CHM hydratase (LigJ) fromSphingomonassp. SYK-6.
169 in this enzyme, suggesting that this nitrile hydratase, like the enzyme from Rhodococcus rhodochrous
170 mponent enzymes of TOC, long-chain enoyl-CoA hydratase, long-chain 3-hydroxyacyl-CoA dehydrogenase, a
171 wn that triosephosphate isomerase, aconitate hydratase, M-protein, nucleoside diphosphate kinase B, a
172 -oxidation mutants showed that the enoyl CoA-hydratase MAOC-1 serves an important role in ascaroside
173 (e.g., isocitrate dehydrogenase and fumarate hydratase) may enhance or mimic the effects of recurrent
174                                      Nitrile hydratase metalloenzymes are unique and important biocat
175 enzyme assigned as (3S)-methylglutaconyl-CoA hydratase (MGCH), which catalyzes the syn-hydration of (
176  In contrast, growth of a putative enoyl-CoA hydratase mutant (DeltaechA) was abolished on short-chai
177 vels in succinate dehydrogenase and fumarate hydratase-mutant tumors, were identified as potent Tet i
178 ing on nitrile metabolizing enzymes: nitrile hydratase (NHase) and amidase versus nitrilase activity.
179                                      Nitrile hydratase (NHase) catalyzes the hydration of nitriles to
180                                      Nitrile hydratase (NHase) is an iron-containing metalloenzyme th
181                                      Nitrile hydratase (NHase) is one of a growing number of enzymes
182 ive site of the non-heme iron enzyme nitrile hydratase (NHase) is studied using sulfur K-edge XAS and
183  catalytic role in the metalloenzyme nitrile hydratase (NHase), a reactive five-coordinate Co(III) th
184 o the catalytic mechanism of Fe-type nitrile hydratases (NHase), the pH and temperature dependence of
185 oth cysteine dioxygenases (CDOs) and nitrile hydratases (NHases), and yet the mechanisms by which sul
186 e a detailed catalytic mechanism for nitrile hydratases (NHases), the pH and temperature dependence o
187 rboxylase (gamma-RSD), and 4-oxalomesaconate hydratase (OMAH).
188 ism caused by deficient activity of fumarate hydratase, one of the constituent enzymes of the Krebs t
189 riments show that the MFP2 2-trans enoyl-CoA hydratase only exhibits activity against long chain (C18
190 on of the resonance Raman spectra of nitrile hydratase prepared at pH 7.3 and 9.0 shows a shift of in
191       The novel family of eukaryotic nitrile hydratases presented in this paper represents a promisin
192 ydratase (probably PrpD), 2-methylisocitrate hydratase (probably PrpD), and 2-methylisocitrate lyase
193  recapitulated by the incubation of fumarate hydratase-proficient cells with cell-permeable fumarate.
194 fer effect on the mechanism of the enoyl-CoA hydratase reaction is discussed.
195 quantitatively assessed from the K(m) in the hydratase reaction, 3 microM, and the K(i), 1.0 microM,
196 , is inactive in the dehydrogenation and the hydratase reactions.
197 xA (Rv1470), trxB (Rv1471), and an enoyl-coA hydratase (Rv1472), indicating a possible role for CtpD
198                                  Two nitrile hydratase sequences from an animal and a plant resolve i
199                   Furthermore, the wild-type hydratase shows a bell-shaped pH dependence of the kcat/
200 , the acyl-CoA ligase SidI and the enoyl-CoA hydratase SidH, linking biosynthesis of mevalonate and T
201 ed to 3-hydroxypropionyl-CoA by acryloyl-CoA hydratase (SPO0147).
202 logy validates the modeling of the enoyl-CoA hydratase structure with the 4-(chlorobenzoyl)-CoA dehal
203 e, and is a fusion of beta and alpha nitrile hydratase subunits.
204 ncer, including the VHL, MET, FLCN, fumarate hydratase, succinate dehydrogenase, TSC1, TSC2, and TFE3
205 art of a consensus sequence in the enoyl-CoA hydratase superfamily, the results presented here provid
206 seven paralogues of the crotonase (enoyl CoA hydratase) superfamily.
207 th the strong sequence homology to enoyl-CoA hydratase support the intramolecular suprafacial transfe
208 s enzyme is a member of the class of nitrile hydratase that contains cobalt.
209 s, including a missense mutation in fumarate hydratase that controls variation in the mitochondrial u
210                                   In nitrile hydratase, the active-site Cys-SOH functions in both iro
211     Like the other cobalt-containing nitrile hydratases, this enzyme is relatively stable, maintainin
212 ompounds where ring-cleavage is achieved via hydratases, this lyase might represent a new ring-openin
213                      Our assay uses fumarate hydratase to convert fumarate to malate and uses oxaloac
214 We have used the similarity of homoaconitate hydratase to isopropylmalate isomerase (serving in leuci
215 ed glutamate residues of rat liver enoyl-CoA hydratase to which Glu119 and Glu139 of the large alpha-
216 onate decarboxylase (4-OD) and vinylpyruvate hydratase (VPH) from Pseudomonas putida mt-2 form a comp
217 otonate tautomerase (4-OT) and vinylpyruvate hydratase (VPH) from the catechol meta-fission pathway a
218 alytically essential in homologous enoyl-CoA hydratases was also essential in CHY1.
219 a coli enolase (EC 4.2.1.11, phosphopyruvate hydratase), which is a component of the RNA degradosome,
220 nzyme activity of an iron-containing nitrile hydratase, which requires a catalytic alphaCys114-SOH in
221 vity changes caused by mutations in fumarate hydratase, which underlie the familial cancer predisposi
222 udes ACMSD and ACMSD-like decarboxylases and hydratases with diverse substrate specificities, many of
223 ease, respectively, in the kcat of enoyl-CoA hydratase without a significant change in the Km value o
224 y an acyl-CoA dehydrogenase and a subsequent hydratase yielding an intermediate with a tertiary hydro

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