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Literature summary for 6.3.4.5 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
Search for more literature for 6.3.4.5

Protein Variants

Protein Variants Comment Organism
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
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
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
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]

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

Molecular Weight [Da]

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

Natural Substrates/ Products (Substrates)

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

Organism

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

Purification (Commentary)

Purification (Comment) Organism
-
 Homo sapiens

Substrates and Products (Substrate)

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

Subunits

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