ライフサイエンスコーパス: urea cycle

1 tase 1, which mediates the first step in the urea cycle.

2 rved enzyme in arginine biosynthesis and the urea cycle.

3 ornithine in the presence of arginase in the urea cycle.

4 into various intermediate metabolites of the urea cycle.

5 ut life to eliminate excess nitrogen via the urea cycle.

6 essential hepatic enzyme that initiates the urea cycle.

7 which controls the rate-limiting step of the urea cycle.

8 y of different enzymatic deficiencies of the urea cycle.

9 se phosphate pathway, and most or all of the urea cycle.

10 n developing embryos, thus avoiding a futile urea cycle.

11 alyzes the initial rate-limiting step of the urea cycle.

12 arginine in the final cytosolic step of the urea cycle.

13 oneogenesis, amino acid homeostasis, and the urea cycle.

14 defect in metabolic pathways, including the urea cycle.

15 by loss of arginine biosynthesis through the urea cycle.

16 BPD group were linked with alteration of the urea cycle.

17 acid metabolism with mild impairment of the urea cycle.

18 involved in the release of auxin and in the urea cycle.

19 athway that clarifies the role of the diatom urea cycle.

20 e mitochondrial fatty acid oxidation and the urea cycle.

21 itted reaction and rate-limiting step in the urea cycle.

22 n pathway and the recently discovered diatom urea cycle.

23 ycles are linked directly with the ornithine-urea cycle.

24 ramitochondrial, rate-limiting enzyme in the urea cycle.

25 vealed alterations in beta-oxidation and the urea cycle.

26 ks, such as the tricarboxylic acid (TCA) and urea cycles.

27 reactions in the tricarboxylic acid and the urea cycles.

28 This index of urea cycle activity also distinguished asymptomatic hete

29 tamine ratio is a sensitive index of in vivo urea cycle activity and correlates with clinical severit

30 Evidence of urea cycle activity restoration was demonstrated by the

31 compromised citric acid cycle flux, enhanced urea cycle activity, and increased amino acid catabolism

32 ed 15NH4 to [15N]urea were used to determine urea-cycle activity in vivo.

33 nge of nitrogenous compounds, and a complete urea cycle, all attributes that allow diatoms to prosper

34 Significant pathways included metabolism of urea cycle/amino group, alanine and aspartate, aspartate

35 aled that dysregulation of the mitochondrial urea cycle and a nucleotide imbalance were associated wi

36 nformation regarding roles and regulation of urea cycle and arginine metabolic enzymes in liver and o

37 turbations in intermediate metabolism in the urea cycle and aspartate-glutamate pathways disrupting m

38 rate-limiting step of arginine synthesis in urea cycle and citrulline-nitric oxide cycle.

39 en, Helicobacter pylori, also has a complete urea cycle and contains the rocF gene encoding arginase

40 present in the liver, is a key enzyme of the urea cycle and eliminates excess ammonia through the exc

41 oyl-phosphate synthetase I (CPSI, related to urea cycle and endogenous nitric oxide production) and c

42 We identified metabolites of BCAA, urea cycle and fatty acid metabolism as biomarkers of HF

43 of mutant cells highlighted purine, arginine/urea cycle and glutamate metabolisms as the most consist

44 d GLUL, all of which are associated with the urea cycle and glutamine biosynthesis.

45 lmalonylation, that inhibited enzymes in the urea cycle and glycine cleavage pathway in MMA.

46 Failure of the urea cycle and hyperammonemia are common in patients wit

47 demonstrates enhanced nitrogen flux into the urea cycle and infusion of (15)N-arginine shows that Arg

48 orter, CITRIN, which is involved in both the urea cycle and malate-aspartate shuttle.

49 rresponding only to metabolites found in the urea cycle and metabolism of amino groups pathway.

50 ses in sphingolipids, indicate that both the urea cycle and nitric oxide pathways are dysregulated at

51 y and the ability to use N via the ornithine-urea cycle and nitric oxide synthase production.

52 nvolved in the glycerol-3-phosphate shuttle, urea cycle and redox reactions were lowered.

53 glutamine from cells required to sustain the urea cycle and the glutamine-glutamate cycle that regene

54 ty acids, dipeptides, and metabolites of the urea cycle and xanthine, steroid, and glutathione metabo

55 d ornithine transcarbamoylase (OTC) from the urea cycle, and enzymes involved in beta-oxidation.

56 ct changes in amino acid serum profiles, the urea cycle, and glycolysis, and attribute the difference

57 he detoxification of ammonia by means of the urea cycle, and in the synthesis of nitric oxide (NO).

58 Arginase I is a component of the urea cycle, and inherited defects in arginase I have del

59 d to the metabolism of steroids, bile acids, urea cycle, and long-chain polyunsaturated fatty acids.

60 in arginase 1 (ARG1), the final step of the urea cycle, and results biochemically in hyperargininemi

61 yll components, nitrate assimilation and the urea cycle, and synthesis of carbohydrate storage compou

62 the flux of (15)N-labeled glutamine into the urea cycle, and the production of urea isotopomers.

63 amides, tryptophan, phospholipids, Krebs and urea cycles, and revealed kidney dysfunction biomarkers.

64 reveals how the enzymes associated with the urea cycle are expressed to ensure proper mass flow of t

65 d ammonia levels due to dysregulation of the urea cycle are known to cause neurologic impairment.

66 indicate that intermediates in the ornithine-urea cycle are particularly depleted and that both the t

67 d-chain amino acid (BCAA) catabolism and the urea cycle are uniporter-regulated pathways.

68 In other tissues that lack a complete urea cycle, arginase regulates cellular arginine and orn

69 an important role in the regulation of extra-urea cycle arginine metabolism by modulating cellular ar

70 ced modulation of hepatic metabolism and the urea cycle as an endogenous mechanism of immunoregulatio

71 egulation of its main metabolic pathway, the urea cycle, as reflected by down-regulation of urea cycl

72 abnormalities in neurotransmitter signaling, urea cycle, aspartate-glutamate metabolism, and glutathi

73 of enzymes related to fatty acid metabolism, urea cycle, cell replication, and mitochondrial function

74 The urea cycle converts ammonia into urea in mammals, amphib

75 Catabolism of Arg through the urea cycle could generate free ammonium in the uninfecte

76 Plasma amino acid analysis suggestive of a urea cycle defect and initiation of a treatment with lac

77 chieved stable therapeutic protection in two urea cycle defect mouse models; a clinically conceivable

78 effect on the hyperammonemia suggesting the urea cycle defect was independent of the aspartate/malat

79 sh the relative contributions of the hepatic urea-cycle defect from those of the NO deficiency to the

80 Urea cycle defects and acute or chronic liver failure ar

81 this technology in the management of severe urea cycle defects could be as a bridging therapy while

82 hown to selectively impact tumors displaying urea cycle defects including a large fraction of hepatoc

83 A-VA deficiency should therefore be added to urea cycle defects, organic acidurias, and pyruvate carb

84 ntial of the system in the context of severe urea cycle defects.

85 Arginase 1 deficiency is a urea cycle disorder associated with hyperargininemia, sp

86 nic aciduria (ASA) is an autosomal recessive urea cycle disorder caused by deficiency of argininosucc

87 en flux through ASS1 in the liver causes the urea cycle disorder citrullinaemia.

88 ewborn mice with a partial deficiency in the urea cycle disorder enzyme, ornithine transcarbamylase (

89 blish an in vitro liver disease model of the urea cycle disorder ornithine transcarbamylase deficienc

90 acetate/benzoate or sodium phenylbutyrate in urea cycle disorder patients has been associated with a

91 ne levels at birth, with negative results of urea cycle disorder testing at the time, along with left

92 type I (CTLN1, OMIM# 215700) is an inherited urea cycle disorder that is caused by an argininosuccina

93 enylbutyrate is a drug used in patients with urea cycle disorder to elicit alternative pathways for n

94 lacetic acid (sodium salt form; treatment of urea cycle disorder).

95 transcarbamylase (OTC) cause the most common urea cycle disorder, OTC deficiency.

96 iciency (OTCD, OMIM 311250), the most common urea cycle disorder, results in impaired synthesis of ci

97 Prompt recognition of a urea-cycle disorder and treatment with both sodium pheny

98 cinic aciduria (ASA), the second most common urea-cycle disorder, and leads to deficiency of both ure

99 amylase deficiency (OTCD) is the most common urea-cycle disorder, characterized by hyperammonemia and

100 w that ASA, in addition to being a classical urea-cycle disorder, is also a model of congenital human

101 anscarbamylase deficiency, the most frequent urea-cycle disorder.

102 Urea cycle disorders (UCD) are inborn errors of metaboli

103 id (PBA), which is approved for treatment of urea cycle disorders (UCDs) as sodium phenylbutyrate (Na

104 Individuals with urea cycle disorders (UCDs) often present with intellect

105 Cells (HepaStem) in pediatric patients with urea cycle disorders (UCDs) or Crigler-Najjar (CN) syndr

106 mended long-term therapy of individuals with urea cycle disorders (UCDs), involve the risk of iatroge

107 tyrate is under development for treatment of urea cycle disorders (UCDs), rare inherited metabolic di

108 butyrate (Buphenyl(R)), a drug used to treat urea cycle disorders and currently in clinical trials fo

109 id butyrate and is approved for treatment of urea cycle disorders and progressive familial intrahepat

110 ying neurological dysfunction that occurs in urea cycle disorders and the only to examine arginase de

111 Urea cycle disorders are a group of inborn errors of hep

112 7 individuals longitudinally followed by the Urea Cycle Disorders Consortium (UCDC) and the European

113 in a natural history study conducted by the Urea Cycle Disorders Consortium, we found that 97% of pl

114 en, common causes of hyperammonaemia include urea cycle disorders or organic acidaemias.

115 eticulous metabolic control in children with urea cycle disorders, because even mildly symptomatic su

116 for reducing plasma ammonia and glutamine in urea cycle disorders, can suppress both proinflammatory

117 um phenylacetate (NaPA), a drug approved for urea cycle disorders, in inhibiting the disease process

118 ug Administration-approved nontoxic drug for urea cycle disorders, in treating the disease process of

119 re liver metabolic function in patients with urea cycle disorders, including ARG1 deficiency.

120 or IEMs (including glutaric aciduria type I, urea cycle disorders, mitochondrial disorders, and lysos

121 Unlike other urea cycle disorders, recurrent hyperammonemia is typica

122 nd Drug Administration-approved drug against urea cycle disorders, upregulates Tregs and protects mic

123 is a new FDA approved drug for management of urea cycle disorders.

124 mmonul, Ucyclyd Pharma) in 299 patients with urea-cycle disorders in whom there were 1181 episodes of

125 treatment of this disorder and other related urea-cycle disorders.

126 ctly regulates OTC activity and promotes the urea cycle during CR, and the results suggest that under

127 ediated cell death, which is associated with urea cycle dysfunction.

128 of specific enzymes in fatty acid oxidation, urea cycle, electron transport, and anti-oxidant pathway

129 rising finding that CPSase III and all other urea cycle enzyme activities are present in muscle of th

130 ions of the relationship between the hepatic urea cycle enzyme activities, the flux of (15)N-labeled

131 ent model was associated with restoration of urea cycle enzyme activity and function, reduced hepatic

132 The urea cycle enzyme arginase (EC 3.5.3.1) hydrolyzes l-arg

133 The urea cycle enzyme argininosuccinate lyase (ASL) enables

134 mutation in the ASL gene, which encodes the urea cycle enzyme argininosuccinate lyase.

135 Downregulation of the urea cycle enzyme argininosuccinate synthase (ASS1) by e

136 In this study, we focused on the urea cycle enzyme argininosuccinate synthetase 1 (ASS1)

137 KL cells express the urea cycle enzyme carbamoyl phosphate synthetase-1 (CPS1

138 Under normal circumstances, the urea cycle enzyme carbamoylphosphate synthetase I (CPS I

139 patic, cardiac and pulmonary disease, and in urea cycle enzyme deficiencies.

140 tency of ornithine transcarbamylase (OTC), a urea cycle enzyme for which loss of catalytic activity c

141 expression of BCAA catabolism genes and the urea cycle enzyme ornithine transcarbamylase.

142 ea cycle, as reflected by down-regulation of urea cycle enzyme protein expression and accumulation of

143 tion of argininosuccinate synthase (ASS1), a urea cycle enzyme suppressed in primary ccRCC, as a cruc

144 caused by deficiency of arginase 1 (ARG1), a urea cycle enzyme that converts arginine to ornithine.

145 Argininosuccinate synthase (ASS1) is a urea cycle enzyme that is essential in the conversion of

146 cohorts of patients with historically lethal urea-cycle enzyme defects.

147 nd they provide a metabolic link between the urea cycle enzymes and pyrimidine synthesis.

148 Depletion of substrates of urea cycle enzymes in ASS1-deficient cancers decreased c

149 ds are major regulators of the expression of urea cycle enzymes in liver.

150 ossibility of packaging the needed amount of urea cycle enzymes in liver.

151 The main aim was to evaluate changes in urea cycle enzymes in NAFLD patients and in two preclini

152 Inborn errors of each of the urea cycle enzymes occur in humans.

153 reduction in the expression and activity of urea cycle enzymes resulting in hyperammonemia, evidence

154 d for genes encoding mitochondrial proteins, urea cycle enzymes, and vacuolar ATPase functions.

155 h the reported "channeling" of substrates by urea cycle enzymes, we hypothesize that the Arg/Cit cycl

156 protein expression patterns of mitochondrial urea cycle enzymes.

157 genes, including Otc and Ass1, which encode urea cycle enzymes.

158 ified by hepatic GS and approximately 35% by urea-cycle enzymes, while approximately 30% is not clear

159 In contrast, the "urea cycle" enzymes in nonhepatic cells are regulated by

160 thesis, which resulted in a 50% reduction in urea cycle flux.

161 Tricarboxylic acid and urea cycle fluxes were also reduced in hypoxia, but phos

162 n enzyme, catalyzing the initial step of the urea cycle for ammonia detoxification and disposal.

163 amino acids, pentose phosphate pathway, and urea cycle, from LRMS and HRMS data.

164 activity in immune cells while sparing liver urea cycle function.

165 is central to the malate-aspartate shuttle, urea cycle, gluconeogenesis and myelin synthesis.

166 zone-specific functions associated with the urea cycle, glutathione synthesis and glutamate synthesi

167 Our data suggest that defects in the urea cycle, glycine, and serine metabolism may be underr

168 Arginase of the Helicobacter pylori urea cycle hydrolyzes L-arginine to L-ornithine and urea

169 xpression and function of key enzymes of the urea cycle in hepatocytes.

170 that interleukin-17 (IL-17) re-programs the urea cycle in keratinocytes increasing polyamines that s

171 neurotoxin that is detoxified mainly by the urea cycle in the liver.

172 high titers of all enzymes of the ornithine-urea cycle in their livers.

173 efficiencies >60% and the restoration of the urea cycle in vitro.

174 he genetically predetermined capacity of the urea cycle--in particular, the efficiency of carbamoyl-p

175 s and potentially neurotoxic ammonia via the urea cycle, including use of only free ammonia as a nitr

176 ting hypercatabolism and upregulation of the urea cycle independent of impaired renal clearance of ni

177 The precursor of nitric oxide is arginine, a urea-cycle intermediate.

178 metabolites remain unchanged from rest; but urea cycle intermediates are increased, likely attributa

179 ted metabolites are generated from ornithine-urea cycle intermediates by the products of genes latera

180 production; (4) increased hepatic and renal urea cycle intermediates suggesting hypercatabolism and

181 athione metabolism) and nitrogen metabolism (urea cycle intermediates) accumulated until the end of t

182 ntrols. A panel of 12 metabolites, including urea cycle intermediates, aromatic amino acids and quino

183 and nucleotides, but an increase in TCA and urea cycle intermediates.

184 ystemic ammonia homeostasis by providing key urea-cycle intermediates and ATP.

185 The urea cycle is comprised of five enzymes but also require

186 The complete urea cycle is expressed in liver and to a small degree a

187 So far, the ornithine-urea cycle is only known for its essential role in the r

188 tagenome was characterized by changes in the urea cycle, L-citrulline biosynthesis and creatinine deg

189 volved in translation, DNA synthesis and the urea cycle like translation initiation factor IF-2, 50S

190 t (Oncorhynchus mykiss), suggesting that the urea cycle may play a physiological role in early develo

191 and identified changes in purine salvage and urea cycle metabolism that may help to limit fumarate ac

192 e model of citrullinemia, an inborn error of urea-cycle metabolism characterized by deficiency of arg

193 ficantly associated with decreased levels of urea cycle metabolites and increased plasma glycine leve

194 included decreases in serotonin derivatives, urea cycle metabolites, and B vitamins.

195 ines; rho = 0.198, p = 0.017), and factor 8 (urea cycle metabolites; rho = - 0.239, p = 4 x 10(-3)),

196 m (spermidine: higher in AD, p = 0.004); (4) urea cycle (N-acetyl glutamate: lower in AD, p < 0.001);

197 expression of several enzymes present in the urea cycle occurs also in many other tissues, where thes

198 use, a model of the X-linked disorder of the urea cycle, ornithine carbamoyltransferase deficiency (O

199 The ornithine-urea cycle (OUC) belongs to the cornerstone of the metab

200 In addition to the urea cycle, our examples include SCH9 and CYR1 (both imp

201 zymes involved in the tricarboxylic acid and urea cycles, oxidative phosphorylation, as well as react

202 ty i.e the Warburg effect (P = 3.62E-03) and urea cycle (P = 7.95E-0.4).

203 activity in liver of the first enzyme in the urea cycle pathway, carbamoyl-phosphate synthetase III (

204 a result of increased purine catabolism and urea cycle pathways.

205 ontrol subjects (ratio = 0.42 +/- 0.06) from urea cycle patients with late (0.17 +/- 0.03) and neonat

206 as an aid to the diagnosis and management of urea cycle patients.

207 genes including those of albumin synthesis, urea cycle, phase I and II metabolic enzymes, and clotti

208 The urea cycle protects the central nervous system from ammo

209 FXR-knockout mice had reduced expression of urea cycle proteins, and accumulated precursors of ureag

210 metabolism: carbamoylphosphate synthetase 1 (urea cycle), pyruvate carboxylase (anaplerosis, gluconeo

211 has been reported that the activities of the urea cycle-related enzymes ornithine carbamoyltransferas

212 s suggest that glycine metabolism and/or the urea cycle represent potentially novel sex-specific mech

213 vivo rates of total body urea synthesis and urea cycle-specific nitrogen flux would correlate with b

214 a variety of related pathways including the urea cycle, TCA cycle, gluconeogenesis, and phosphatidyl

215 and is detoxified by the five enzymes of the urea cycle that are expressed within the liver.

216 Arginase, a key metalloenzyme of the urea cycle that converts L-arginine into L-ornithine and

217 yzes the entry and rate-limiting step in the urea cycle, the pathway by which mammals detoxify ammoni

218 The diatom ornithine-urea cycle therefore represents a key pathway for anaple

219 h a reliance on nitrate assimilation and the urea cycle to help fuel energy production.

220 d whose expression, similar to that of other urea cycle (UC) components, was high in liver and varied

221 The expression of the urea cycle (UC) proteins is dysregulated in multiple can

222 d H2/CO2 (compared to fructose) point to the urea cycle, uptake and degradation of peptides and amino

223 activity of OTC, a rate-limiting step in the urea cycle was also dose dependently repressed.

224 show that the exosymbiont-derived ornithine-urea cycle, which is similar to that of metazoans but is

225 fferent enzymatic deficiencies affecting the urea cycle while consuming a low protein diet.