ライフサイエンスコーパス: urea cycle

1 essential hepatic enzyme that initiates the urea cycle.

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

3 y of different enzymatic deficiencies of the urea cycle.

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

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

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

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

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

9 e mitochondrial fatty acid oxidation and the urea cycle.

10 n pathway and the recently discovered diatom urea cycle.

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

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

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

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

15 rved enzyme in arginine biosynthesis and the urea cycle.

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

17 into various intermediate metabolites of the urea cycle.

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

19 reactions in the tricarboxylic acid and the urea cycles.

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

21 This index of urea cycle activity also distinguished asymptomatic hete

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

54 Urea cycle defects and acute or chronic liver failure ar

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

83 Unlike other urea cycle disorders, recurrent hyperammonemia is typica

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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