6.3.4.5: argininosuccinate synthase
This is an abbreviated version!
For detailed information about argininosuccinate synthase, go to the full flat file.
Word Map on EC 6.3.4.5
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6.3.4.5
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ornithine
-
citrullinemia
-
arginase
-
ammonia
-
hyperammonemia
-
deiminase
-
transcarbamylase
-
l-arginine
-
pegylated
-
auxotrophic
-
adi-peg20
-
carbamyl
-
adult-onset
-
coma
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argininosuccinase
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carbamoylphosphate
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carbamoyltransferase
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citrin
-
tropoelastin
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urea-cycle
-
consciousness
-
medicine
-
ureogenesis
-
elastogenesis
-
ornithine-urea
-
elastin-derived
-
jararaca
-
coinduction
-
bothrops
-
n-acetylglutamate
-
drug development
-
molecular biology
- 6.3.4.5
- ornithine
- citrullinemia
- arginase
- ammonia
- hyperammonemia
- deiminase
-
transcarbamylase
- l-arginine
-
pegylated
-
auxotrophic
-
adi-peg20
-
carbamyl
-
adult-onset
- coma
- argininosuccinase
- carbamoylphosphate
-
carbamoyltransferase
-
citrin
- tropoelastin
-
urea-cycle
-
consciousness
- medicine
-
ureogenesis
-
elastogenesis
-
ornithine-urea
-
elastin-derived
- jararaca
-
coinduction
-
bothrops
- n-acetylglutamate
- drug development
- molecular biology
Reaction
Synonyms
ARGG, Arginine succinate synthetase, argininosuccinate synthase, argininosuccinate synthase 1, Argininosuccinate synthetase, Argininosuccinic acid synthetase, arininosuccinate synthetase, AS, ASS, ASS1, Citrulline--aspartate ligase, Citrulline-aspartate ligase, EAS, Elastin-binding protein, PyARG1, Synthetase, argininosuccinate, tAsS
ECTree
6.3.4.5 argininosuccinate synthaseAdvanced search results
results (
results (Substrates Products
Substrates Products on EC 6.3.4.5 - argininosuccinate synthase
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SUBSTRATE 
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
ATP + L-citrulline + 3-nitro-2-aminopropanoate
?
-
60% of the maximal activity with Asp
-
-
?
dATP + L-citrulline + L-Asp
dAMP + diphosphate + L-argininosuccinate
-
2.6% as active as ATP
-
-
?
ATP + citrulline + aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
-
?
ATP + citrulline + aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
-
?
ATP + citrulline + aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
assay at pH 7.8, 37°C, reaction stopped by addition of molybdate buffer
-
-
?
ATP + citrulline + aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
-
?
ATP + L-citrulline + L-Asp
?
-
mechanism to recycle citrulline, formed in the nervous system via nitric oxide synthetase, EC 1.14.13.39, activity, back to the nitric oxide precursor, L-Arg
-
-
?
ATP + L-citrulline + L-Asp
?
-
anabolic function in biosynthesis of Arg and catabolic function as the first enzyme of citrulline utilization as nitrogen source
-
-
?
ATP + L-citrulline + L-Asp
?
-
anabolic function in biosynthesis of Arg and catabolic function as the first enzyme of citrulline utilization as nitrogen source
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
D-Asp is inactive as substrate
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
argininosuccinate synthase catalyzes the rate-limiting step in the recycling of citrulline to arginine, which in endothelial cells, is tighly coupled to the production of nitric oxide. The upstream open reading frame found in the extended, overlapping 5'-untranslated region of argininosuccinate synthase mRNA species suppresses endothelial argininosuccinate expression providing a novel mechanism for regulating endothelial NO production by limiting the availability of arginine
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
argininosuccinate synthase expression is required to maintain nitric oxide production and cell viability in aortic endothelial cells
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
IL-1beta stimulates argininosuccinate synthetase gene expression through NF-kappaB in Caco-2 cells
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
the substrate for NO synthesis, L-arginine can be regenerated from the L-citrulline the coproduct of nitric-oxide synthase. This requires the sequential action of two enzymes, argininosuccinate synthetase and argininosuccinate lyase
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
-
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
r
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
r
-
?
ATP + L-citrulline + L-Asp
AMP + diphosphate + L-argininosuccinate
-
r
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
argininosuccinate synthase is essential for endothelial nitric oxide production, vascular NO production is regulated by dynamic argininosuccinate synthase phosphorylation
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
the caveolar-localized endothelial argininosuccinate synthase catalyzes the rate-limiting step of the citrulline-NO cycle, involving not only the regulation of endothelial nitric oxide synthase, but also regulation of cycle, overview
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
citrulline-aspartate ligase is the rate-limiting enzyme in the synthesis of the amino acid arginine. Type I citrullinaemia is a disorder caused by mutations in the ASS gene leading to reduced or abolished activity of the ASS enzyme
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
rate-limiting enzyme in nitric oxide synthesis, negative feedback mechanism, overview
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
the key enzyme in the urea cycle participates in many metabolic processes including arginine biosynthesis and the citrulline-nitric oxide cycle
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
substrate binding structures, overview
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
the enzyme plays an important role as the rate-limiting step required for converting L-citrulline to argininosuccinate to provide arginine
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
the enzyme plays an important role as the rate-limiting step required for converting L-citrulline to argininosuccinate to provide arginine
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
rate-limiting enzyme of the urea cycle
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
Sulfolobus solfataricus lacks ornithine acetyltransferase and thus forms N-acetylglutamate exclusively via the energetically less favourable reaction catalysed by N-acetylglutamate synthase, investing 1 mol of acetyl CoA per mol of N-acetyl intermediate synthesized
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
Sulfolobus solfataricus lacks ornithine acetyltransferase and thus forms N-acetylglutamate exclusively via the energetically less favourable reaction catalysed by N-acetylglutamate synthase, investing 1 mol of acetyl CoA per mol of N-acetyl intermediate synthesized
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
catalyzes the coupling reaction between citrulline and arginine to form argininosuccinic acid
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
the rate-limiting enzyme for converting citrulline to arginine
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
ATP induces conformational changes in the nucleotide binding domain, each monomer of tetrameric EAS consists of a nucleotide binding domain and a novel catalytic/multimerization domain, detailed catalytic mechanism via adenylated citrulline intermediate, active site structure
-
r
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
ATP-dependent ligation of citrulline and aspartate
-
r
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
penultimate step in the biosnthesis of arginine, rate-limiting enzyme of both the urea and arginine-citrulline cycles
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
catalyzes the coupling reaction between citrulline and arginine to form argininosuccinic acid
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
the rate-limiting enzyme for converting citrulline to arginine
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
catalyzes the coupling reaction between citrulline and arginine to form argininosuccinic acid
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
the rate-limiting enzyme for converting citrulline to arginine
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
mediates transformation of L-citrulline to L-arginine in the ureum cycle in hepatocytes, first step in L-citrulline recycling pathway
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
-
-
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
ATP-dependent condensation of citrulline with aspartate, detailed mechanism via citrullyl-AMP intermediate, stereochemistry of catalysis, no conformational change upon binding of ATP, active site structure
-
r
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
ATP-dependent condensation of citrulline with aspartate, no conformational change upon binding of substrates, mechanism via citrullyl-AMP intermediate, active site structure
-
r
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
-
second step of urea cycle
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
seventh step of the arginine biosynthesis, second step of the urea cycle
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
Thermus thermophilus HB8 / ATCC 27634 / DSM 579
-
ATP-dependent condensation of citrulline with aspartate, no conformational change upon binding of substrates, mechanism via citrullyl-AMP intermediate, active site structure
-
r
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
Thermus thermophilus HB8 / ATCC 27634 / DSM 579
-
second step of urea cycle
-
?
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
Thermus thermophilus HB8 / ATCC 27634 / DSM 579
ATP-dependent condensation of citrulline with aspartate, detailed mechanism via citrullyl-AMP intermediate, stereochemistry of catalysis, no conformational change upon binding of ATP, active site structure
-
r
ATP + L-citrulline + L-aspartate
AMP + diphosphate + L-argininosuccinate
Thermus thermophilus HB8 / ATCC 27634 / DSM 579
seventh step of the arginine biosynthesis, second step of the urea cycle
-
?
additional information
?
-
-
citrullinemia is an autosomal recessive disease caused by a deficiency of argininosuccinate synthetase
-
-
?
additional information
?
-
-
adult-type citrullinemia with neuro-psychiatric symptoms of late onset, 24-28 years old, caused by argininosuccinate synthetase abnormalities
-
-
?
additional information
?
-
-
rate-limiting enzyme in the metabolic pathway leading from L-citrulline to L-arginine, urea cycle
-
?
additional information
?
-
-
factors like diets, hormones and pro-inflammatory stimuli regulate ASS gene expression primarily at the transcription level
-
-
?
additional information
?
-
in mammals, the urea-cycle enzymes ASS and argininosuccinatelyase are part of an additional pathway together with the enzyme nitric oxide synthase, forming the arginine-citrulline cycle
-
-
?
additional information
?
-
-
in mammals, the urea-cycle enzymes ASS and argininosuccinatelyase are part of an additional pathway together with the enzyme nitric oxide synthase, forming the arginine-citrulline cycle
-
-
?
additional information
?
-
-
pancreatic cancer cell lines deficient in argininosuccinate synthetase are sensitive to arginine deprivation by arginine deiminase
-
-
?
additional information
?
-
the enzyme interacts and cooperates with NADPH sensor protein, HSCARG, in downregulation of nitric oxide synthesis, the enzyme interacts with the dimerization region of HSCARG, amino acid residues 153-189, HSCARG regulation of AS activity is crucial for maintaining the intracellular balance between redox state and nitric oxide levels, overview
-
-
?
additional information
?
-
-
involved in L-arginine synthesis from L-citrulline, urea cycle, rate-limiting enzyme for high output NO generation, inducible enzyme, AS synthesis is more strictly regulated at the posttranscriptional than the transcriptional level in vivo
-
?
additional information
?
-
-
urea cycle, enzyme of citrulline-arginine recycling, which is important for high output production of NO in activated microglial cells, inducible enzyme
-
?
additional information
?
-
-
ASS binds to and inactivates lipopolysaccharide and lipid A, overview
-
-
?
additional information
?
-
-
involved in the recycling of L-citrulline to L-arginine
-
?
additional information
?
-
-
urea cycle, enzyme of citrulline-arginine recycling, which is important for high output production of NO in activated microglial cells, inducible enzyme
-
?
