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E296Q
-
retains only 0.001% residual activity
S384A
-
catalytic efficiency is decreased by an order of magnitude in comparison to wild-type delta II crystallin
Y323F
-
catalytic efficiency is decreased by an order of magnitude in comparison to wild-type delta II crystallin
A41G/I43M
-
mutant without enzyme activity
D115A
-
mutant with 45% enzyme activity
D115E
-
mutant with 42.6% enzyme activity
E115D
-
mutant without enzyme activity
I143M
-
mutant without enzyme activity
M9F/E115D
-
mutant with 3.3% enzyme activity
M9W
-
mutant with 2.0% enzyme activity
M9W/E115D
-
mutant with 17% enzyme activity
M9W/V14S
-
mutant with 1.9% enzyme activity
M9Y/E115D
-
mutant with 5.5% enzyme activity
TRUN1-8
-
mutant with 37% enzyme activity
TRUN1-9
-
mutant with 2.1% enzyme activity
W9A
-
mutant with 1.9% enzyme activity
W9F
-
mutant with 11.3% enzyme activity
W9M
-
mutant with 1.0% enzyme activity
W9R
-
mutant with 3% enzyme activity
W9Y
-
mutant with 49.5% enzyme activity
A398D
-
no activity, 15.55 of wild-type activity if coexpressed with Q286R
D31N
missense mutation found in patients with late onset of argininosuccinic aciduria, residual activity
D87G
-
no activity, 35.2% of wild-type activity if coexpressed with Q286R
E189G
the mutant enzyme decreased enzyme efficiency (78% of wild-type), no significant decrease in Km-value, displays thermal instability
E73K
missense mutation found in patient with neonatal onset of argininosuccinic aciduria, enzyme inactive
I100T
the mutant enzyme decreased enzyme efficiency (61% of wild-type), no significant decrease in Km-value, displays thermal instability
K288R
-
132% of wild-type activity
M360T
-
no actiivty, 10% of wild-type activity if coexpressed with Q286R
M382R
missense mutation found in patient with neonatal onset of argininosuccinic aciduria, residual activity
R12Q
-
6% of wild-type kcat
R182Q
missense mutation found in patient with late onset of argininosuccinic aciduria, enzyme inactive
R186Q
missense mutation found in patient with neonatal onset of argininosuccinic aciduria, residual activity
R297Q
missense mutation found in patient with late onset of argininosuccinic aciduria, residual activity
R379C
no significant decrease in Km-value, displays thermal instability
R456W missense mutation found in patient with neonatal onset of argininosuccinic aciduria
residual activity
R95C
the mutant enzyme decreased enzyme efficiency (32% of wild-type), no significant decrease in Km-value, displays thermal instability
C441A
striking decrease in the enzymatic activity, while retaining the overall secondary to quaternary structure of the protein
C441S
significant increase in activity, as compared to the wild-type enzyme, the mutant enzyme has higher thermal stability and maintains significant activity at high temperatures. The mutant shows a marked increase in enzymatic activity as it lacks this cysteine dependent feedback inhibition
C441A
-
striking decrease in the enzymatic activity, while retaining the overall secondary to quaternary structure of the protein
-
C441S
-
significant increase in activity, as compared to the wild-type enzyme, the mutant enzyme has higher thermal stability and maintains significant activity at high temperatures. The mutant shows a marked increase in enzymatic activity as it lacks this cysteine dependent feedback inhibition
-
R140L
-
naturally occuring mutation of variant OsASL1.1, which renders the enzyme catalytically inactive
Q286R
-
3% of wild-type activity
Q286R
-
frequently complementing allele, 2% of wild-type kcat
Q286R
missense mutation found in patient with neonatal onset of argininosuccinic aciduria, enzyme inactive
R113Q
missense mutation found in patient with neonatal onset of argininosuccinic aciduria, enzyme inactive
R113Q
-
site-directed mutagenesis, the R113Q mutation abolishes the catalytic activity of the enzyme without affecting its protein stability
R236W
missense mutation found in patient with neonatal onset of argininosuccinic aciduria, enzyme inactive
R236W
-
site-directed mutagenesis, catalytically inactive mutant, that is structurally intact
V178M
missense mutation combined with mutation R186Q on the other allele found in patient with neonatal onset of argininosuccinic aciduria, enzyme activity reduced compared to wild-type
V178M
no significant reduced Km-value, displays thermal instability
V335L
missense mutation found in patients with late onset of argininosuccinic aciduria, enzyme activity reduced compared to wild-type
V335L
the mutant enzyme decreased enzyme efficiency (48% of wild-type), no significant decrease in Km-value, displays thermal instability
additional information
-
double loop mutant DLM, enzymatically inactive
additional information
-
the mutations E86A and R113W identified on separate alleles are most likely to determine ASL-deficiency in a severely affected patient with neonatal onset of the disease
additional information
-
expression of mutant ASL proteins in Escherichia coli. The known classical p.Q286R, the novel classical p.K315E and the known mutations p.I100T, p.E189G and p.R385C, which all have been linked to a mild phenotype of argininosuccinic aciduria, show no significant residual activity. There is some enzyme activity detected with the p.V178M (5% of wild-type), and p.R379C (10% of wild-type) mutations in which Km values for argininosuccinic acid differs significantly from the wild-type ASL protein
additional information
-
construction of an asl gene-deleted mutant strain S2308DELTAASL by allelic exchange involving insertion of a chloramphenicol resistance cassette in a BamHI site
additional information
-
generation of enterocyte-specific knockout mice of the enzyme Asl by intercrossing Aslflox/flox mice with transgenic mice expressing Cre recombinase under the control of the villin promoter. Emgineered mice are grossly indistinguishable from their littermate Aslflox/flox controls and exhibit similar growth curves and life span. Enterocytes isolated from mutant mice demonstrate an 80% reduction in mRNA expression of the enzyme
additional information
-
construction of an asl gene-deleted mutant strain S2308DELTAASL by allelic exchange involving insertion of a chloramphenicol resistance cassette in a BamHI site
-
additional information
-
isolation of the osred1, i.e. Oryza sativa root elongation defect, mutant from a short root-phenotype Tos-17 transposon insertion line, NE7046
additional information
-
knock-down of enzyme Asl in IEC-6 cells using lentivirus encoding shRNA targeting the enzyme. Loss of the enzyme impairs the migration of IEC-6 cells during conditions of stress
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Nitro analogs of substrates for argininosuccinate synthetase and argininosuccinate lyase
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1984
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Kinetic mechanism of bovine liver argininosuccinate lyase
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221
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Human liver arginiosuccinase purification and partial characterization
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Argininosuccinate lyase: purification and characterization from human liver
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Homo sapiens
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Urea as a selective inhibitor of argininosuccinate lyase
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On the role of substrate and GTP in the regulation of argininosuccinase activity
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Expression of the chicken delta 2-crystallin gene in mouse cells: evidence for encoding of argininosuccinate lyase
Gene
99
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Gallus gallus
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Homo sapiens
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Screening and kinetic analysis of delta-crystallins with endogenous argininosuccinate lyase activity in the lenses of vertebrates
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Anas platyrhynchos, Anser sp., Gallus gallus
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delta-Crystallin is more thermostable than mammalian argininosuccinate lyase
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Anas platyrhynchos, Bos taurus, Sus scrofa
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Expression of duck lens delta-crystallin cDNAs in yeast and bacterial hosts
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Anas platyrhynchos
-
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Lee, H.J.; Lin, C.C.; Chiou, S.H.; Chang, G.G.
Characterization of the multiple forms of duck lens delta-crystallin with endogenous argininosuccinate lyase activity
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314
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Anas platyrhynchos
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Inactivation of the endogenous argininosuccinate lyase activity of duck delta-crystallin by modification of an essential histidine residue with diethyl pyrocarbonate
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Anas platyrhynchos
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Anas platyrhynchos, Homo sapiens
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Three-dimensional structure of the argininosuccinate lyase frequently complementing allele Q286R
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Homo sapiens
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Mechanisms for intragenic complementation at the human argininosuccinate lyase locus
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Homo sapiens
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Braissant, O.; Gotoh, T.; Loup, M.; Mori, M.; Bachmann, C.
L-arginine uptake, the citrulline-NO cycle and arginase II in the rat brain: an in situ hybridization study
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Rattus norvegicus
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Homo sapiens
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Troshina, O.; Hansel, A.; Lindblad, P.
Cloning, characterization, and functional expression in Escherichia coli of argH encoding argininosuccinate lyase in the cyanobacterium Nostoc sp. strain PCC 73102
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Nostoc sp. (Q9LAE5), Nostoc sp.
brenda
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L-citrulline recycling by argininosuccinate synthetase and lyase in rat gastric fundus
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2002
Rattus norvegicus
brenda
Cohen-Kupiec, R.; Kupiec, M.; Sandbeck, K.; Leigh, J.A.
Functional conservation between the argininosuccinate lyase of the archaeon Methanococcus maripaludis and the corresponding bacterial and eukaryal genes
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173
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Methanococcus maripaludis
brenda
Sampaleanu, L.M.; Yu, B.; Howell, P.L.
Mutational analysis of duck delta 2 crystallin and the structure of an inactive mutant with bound substrate provide insight into the enzymatic mechanism of argininosuccinate lyase
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Anas platyrhynchos
brenda
Latasa, M.U.; Carretero, M.V.; Garcia-Trevijano, E.R.; Torres, L.; Mato, J.M.; Avila, M.A.
Identification of argininosuccinate lyase as a hypoxia-responsive gene in rat hepatocytes
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Rattus norvegicus
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Two novel mutations (E86A, R113W) in argininosuccinate lyase deficiency and evidence for highly variable splicing of the human argininosuccinate lyase gene
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23
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Homo sapiens
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Arginosuccinate synthetase, arginosuccinate lyase and NOS in canine gastroinestinal tract
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2000
Canis lupus familiaris
brenda
Bhaumik, P.; Koski, M.K.; Bergmann, U.; Wierenga, R.K.
Structure determination and refinement at 2.44 A resolution of argininosuccinate lyase from Escherichia coli
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Escherichia coli
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A duck delta1 crystallin double loop mutant provides insight into residues important for argininosuccinate lyase activity
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Anas sp.
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Tsai, M.; Koo, J.; Howell, P.L.
Recovery of argininosuccinate lyase activity in duck delta1 crystallin
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Anas sp.
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Molecular cloning and analysis of the argH gene encoding argininosuccinate lyase from Corynebacterium glutamicum
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Corynebacterium glutamicum
-
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Bizzoco, E.; Vannucchi, M.G.; Faussone-Pellegrini, M.S.
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Rattus norvegicus
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Wagemaker, M.J.; Eastwood, D.C.; van der Drift, C.; Jetten, M.S.; Burton, K.; Van Griensven, L.J.; Op den Camp, H.J.
Argininosuccinate synthetase and argininosuccinate lyase: two ornithine cycle enzymes from Agaricus bisporus
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Bizzoco, E.; Faussone-Pellegrini, M.S.; Vannucchi, M.G.
Activated microglia cells express argininosuccinate synthetase and argininosuccinate lyase in the rat brain after transient ischemia
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Rattus norvegicus
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Arabidopsis thaliana
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Recombinant human arginase inhibits proliferation of human hepatocellular carcinoma by inducing cell cycle arrest
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Mitchell, S.; Ellingson, C.; Coyne, T.; Hall, L.; Neill, M.; Christian, N.; Higham, C.; Dobrowolski, S.F.; Tuchman, M.; Summar, M.; Summar, M.
Genetic variation in the urea cycle: a model resource for investigating key candidate genes for common diseases
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Homo sapiens
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Changes of activity and mRNA expression of urea cycle enzymes in the liver of developing Holstein calves
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Bos taurus
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Feng, J.F.; Chen, T.M.; Wen, Y.A.; Wang, J.; Tu, Z.G.
Study of serum argininosuccinate lyase determination for diagnosis of liver diseases
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2008
Homo sapiens
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Laberge, T.; Walsh, P.J.; McDonald, M.D.
Effects of crowding on ornithine-urea cycle enzyme mRNA expression and activity in gulf toadfish (Opsanus beta)
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Levillain, O.; Rabier, D.; Duclos, B.; Gaudreau, P.; Vinay, P.
L-Arginine metabolism in dog kidney and isolated nephron segments
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2008
Canis lupus familiaris
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Bringel, F.; Hammann, P.; Kugler, V.; Arsene-Ploetze, F.
Lactobacillus plantarum response to inorganic carbon concentrations: PyrR2-dependent and -independent transcription regulation of genes involved in arginine and nucleotide metabolism
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2008
Lactiplantibacillus plantarum
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Eastwood, D.C.; Challen, M.P.; Zhang, C.; Jenkins, H.; Henderson, J.; Burton, K.S.
Hairpin-mediated down-regulation of the urea cycle enzyme argininosuccinate lyase in Agaricus bisporus
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Nagasaka, H.; Tsukahara, H.; Yorifuji, T.; Miida, T.; Murayama, K.; Tsuruoka, T.; Takatani, T.; Kanazawa, M.; Kobayashi, K.; Okano, Y.; Takayanagi, M.
Evaluation of endogenous nitric oxide synthesis in congenital urea cycle enzyme defects
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2009
Homo sapiens
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Chen, G.F.; Baylis, C.
In vivo renal arginine release is impaired throughout development of chronic kidney disease
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298
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2010
Rattus norvegicus
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Swamy, M.; Sirajudeen, K.N.; Chandran, G.
Nitric oxide (NO), citrulline-NO cycle enzymes, glutamine synthetase, and oxidative status in kainic acid-mediated excitotoxicity in rat brain
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2009
Rattus norvegicus
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Trevisson, E.; Burlina, A.; Doimo, M.; Pertegato, V.; Casarin, A.; Cesaro, L.; Navas, P.; Basso, G.; Sartori, G.; Salviati, L.
Functional complementation in yeast allows molecular characterization of missense argininosuccinate lyase mutations
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Mercimek-Mahmutoglu, S.; Moeslinger, D.; Haeberle, J.; Engel, K.; Herle, M.; Strobl, M.W.; Scheibenreiter, S.; Muehl, A.; Stoeckler-Ipsiroglu, S.
Long-term outcome of patients with argininosuccinate lyase deficiency diagnosed by newborn screening in Austria
Mol. Genet. Metab.
100
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2010
Homo sapiens
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Neill, M.A.; Aschner, J.; Barr, F.; Summar, M.L.
Quantitative RT-PCR comparison of the urea and nitric oxide cycle gene transcripts in adult human tissues
Mol. Genet. Metab.
97
121-127
2009
Homo sapiens
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