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Literature summary for 4.3.2.1 extracted from

  • Erez, A.; Nagamani, S.C.; Shchelochkov, O.A.; Premkumar, M.H.; Campeau, P.M.; Chen, Y.; Garg, H.K.; Li, L.; Mian, A.; Bertin, T.K.; Black, J.O.; Zeng, H.; Tang, Y.; Reddy, A.K.; Summar, M.; OBrien, W.E.; Harrison, D.G.; Mitch, W.E.; Marini, J.C.; Aschner, J.L.; Bryan, N.S.; Lee, B.
    Requirement of argininosuccinate lyase for systemic nitric oxide production (2011), Nat. Med., 17, 1619-1626.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 4.3.2.1

Application

Application Comment Organism
medicine the enzyme may serve as a target for manipulating NO production and treatment of NO-related diseases Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
in vitro overexpression of ASL enzyme mutant R236W in COS7 cells andprimary fibroblasts Homo sapiens

Protein Variants

Protein Variants Comment Organism
R113Q site-directed mutagenesis, the R113Q mutation abolishes the catalytic activity of the enzyme without affecting its protein stability Homo sapiens
R236W site-directed mutagenesis, catalytically inactive mutant, that is structurally intact Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
cytoplasm
-
 Mus musculus 5737
-
cytoplasm
-
 Homo sapiens 5737
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
2-(Nomega-L-arginino)succinate Mus musculus
-
 fumarate + L-arginine
-
 r
2-(Nomega-L-arginino)succinate Homo sapiens
-
 fumarate + L-arginine
-
 r

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
 Homo sapiens
-
liver
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2-(Nomega-L-arginino)succinate
-
 Mus musculus fumarate + L-arginine
-
 r
2-(Nomega-L-arginino)succinate
-
 Homo sapiens fumarate + L-arginine
-
 r

Synonyms

Synonyms Comment Organism
ASL
-
 Mus musculus
ASL
-
 Homo sapiens

General Information

General Information Comment Organism
malfunction argininosuccinic aciduria, ASA, MIM 207900, is the second most common human urea cycle disorder and is caused by deficiency of argininosuccinate lyase. Subjects with ASA disease cannot generate arginine from citrulline. Loss of the enzyme also leads to reduced NO synthesis due to decreased endogenous arginine synthesis as well as reduced utilization of extracellular arginine for NO production in humans Homo sapiens
malfunction loss of the enzyme leads to reduced NO synthesis due to decreased endogenous arginine synthesis as well as reduced utilization of extracellular arginine for NO production in mice. Enzyme hypomorphic mice show global NO deficiency and evidence of multi-organ dysfunction, phenotypes, hypomorphic mouse model of enzyme deficiency, overview Mus musculus
additional information Asl enzyme-dependent nitric oxide synthase complex Mus musculus
additional information Asl enzyme-dependent nitric oxide synthase complex Homo sapiens
physiological function the enzyme has a structural function in addition to its catalytic activity. The enzyme plays a more central role in cellular arginine utilization for NO synthesis beyond intracellular recycling of citrulline into arginine Mus musculus
physiological function the enzyme has a structural function in addition to its catalytic activity. The enzyme plays a more central role in cellular arginine utilization for NO synthesis beyond intracellular recycling of citrulline into arginine Homo sapiens