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Literature summary extracted from

  • Shaheen, N.; Kobayashi, K.; Terazono, H.; Fukushige, T.; Horiuchi, M.; Saheki, T.
    Characterization of human wild-type and mutant argininosuccinate synthetase proteins expressed in bacterial cells (1994), Enzyme Protein, 95, 251-264.
    View publication on PubMed

Protein Variants

EC Number Protein Variants Comment Organism
6.3.4.5 A192V mutant G280R has no ASS activity, mutants A192V, R272C, and R304W have low ASS activity and abnormal kinetics. A higher Km for citrulline than in wild-type enzyme is obtained with mutants A192V, R272C, and R304W Homo sapiens
6.3.4.5 G280R mutant G280R has no ASS activity, mutants A192V, R272C, and R304W have low ASS activity and abnormal kinetics. A higher Km for citrulline than in wild-type enzyme is obtained with mutants A192V, R272C, and R304W Homo sapiens
6.3.4.5 R272C mutant G280R has no ASS activity, mutants A192V, R272C, and R304W have low ASS activity and abnormal kinetics. A higher Km for citrulline than in wild-type enzyme is obtained with mutants A192V, R272C, and R304W Homo sapiens
6.3.4.5 R304W mutant G280R has no ASS activity, mutants A192V, R272C, and R304W have low ASS activity and abnormal kinetics. A higher Km for citrulline than in wild-type enzyme is obtained with mutants A192V, R272C, and R304W Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
6.3.4.5 additional information
-
additional information
-
Homo sapiens
6.3.4.5 0.027
-
Asp wild-type enzyme Homo sapiens
6.3.4.5 0.051
-
ATP wild-type enzyme Homo sapiens
6.3.4.5 0.056
-
citrulline wild-type enzyme Homo sapiens
6.3.4.5 0.081
-
ATP mutant enzyme A192V Homo sapiens
6.3.4.5 0.14
-
ATP mutant R272C Homo sapiens
6.3.4.5 0.44
-
ATP mutant R304W Homo sapiens
6.3.4.5 1.5
-
Asp mutant A192V Homo sapiens
6.3.4.5 4.2
-
citrulline mutant R272C Homo sapiens
6.3.4.5 4.3
-
Asp mutant R272C Homo sapiens
6.3.4.5 15
-
citrulline mutant A192V Homo sapiens
6.3.4.5 56
-
citrulline mutant R304W Homo sapiens
6.3.4.5 190
-
citrulline mutant R304W Homo sapiens

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
6.3.4.5 46000
-
4 * 46000, SDS-PAGE Homo sapiens
6.3.4.5 180000
-
gel filtration Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
6.3.4.5 ATP + L-citrulline + L-Asp Homo sapiens urea-cycle enzyme ?
-
?
6.3.4.5 additional information Homo sapiens citrullinemia is an autosomal recessive disease caused by a deficiency of argininosuccinate synthetase ?
-
?

Organism

EC Number Organism UniProt Comment Textmining
6.3.4.5 Homo sapiens
-
wild-type and mutant enzymes
-
6.3.4.5 Homo sapiens
-
wild-type and mutant enzyme
-
6.3.4.5 Homo sapiens
-
citrullinemia is an autosomal recessive disease caused by a deficiency of argininosuccinate synthetase
-

Purification (Commentary)

EC Number Purification (Comment) Organism
6.3.4.5
-
Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
6.3.4.5 ATP + citrulline + aspartate
-
Homo sapiens AMP + diphosphate + 2-(Nomega-L-arginino)succinate
-
?
6.3.4.5 ATP + L-citrulline + L-Asp
-
Homo sapiens AMP + diphosphate + L-argininosuccinate
-
?
6.3.4.5 ATP + L-citrulline + L-Asp urea-cycle enzyme Homo sapiens ?
-
?
6.3.4.5 additional information citrullinemia is an autosomal recessive disease caused by a deficiency of argininosuccinate synthetase Homo sapiens ?
-
?

Subunits

EC Number Subunits Comment Organism
6.3.4.5 tetramer 4 * 46000, SDS-PAGE Homo sapiens