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Unknown Unknown Unknown Unknown Unknown Asparagine synthetase [glutamine- hydrolyzing] Adenylosuccinate lyase Isoaspartyl peptidase/L- asparaginase Argininosuccinate lyase Aspartoacylase Asparagine-- tRNA ligase, cytoplasmic Adenylosuccinate synthetase isozyme 1 L-amino-acid oxidase Aspartate--tRNA ligase, cytoplasmic Argininosuccinate synthase CAD protein N- Acetylaspartate synthetase 4-aminobutyrate aminotransferase, mitochondrial Glutamate decarboxylase 1 D-aspartate oxidase L-Aspartic acid Citrulline β-Alanine D-Aspartic acid D-Aspartic acid Oxalacetic acid L-Aspartic acid β-Alanine Oxalacetic acid ATP L-Glutamine H2O AMP PPi L-Asparagine L-Glutamic acid Fumaric acid AMP H2O NH3 Argininosuccinic acid L-Arginine N-Acetyl-L-aspartic acid H2O Acetic acid ATP AMP PPi GTP Inosinic acid GDP Pi Adenylsuccinic acid H2O O2 NH3 H2O2 ATP AMP PPi ATP AMP PPi Pi Ureidosuccinic acid Acetyl-CoA CoA Oxoglutaric acid Malonic semialdehyde L-Glutamic acid CO2 H2O O2 NH3 H2O2 Zinc (II) ion Magnesium FAD Pyridoxal 5'-phosphate FAD tRNA(Asn) L- Asparaginyl- tRNA(Asn) tRNA(Asp) L- Aspartyl- tRNA(Asp) Histidine Metabolism Urea Cycle Urea Cycle Arginine and Proline Metabolism beta-Alanine Metabolism Glycine and Serine Metabolism Nicotinate and Nicotinamide Metabolism Pyrimidine Metabolism Pantothenate and CoA Biosynthesis Pyridoxal 5'-phosphate Oxalacetic acid Carbamoyl phosphate Zinc (II) ion Intracellular Space Extracellular Space Mitochondrion Lysosome Peroxisome Symptoms of the disorder include microcephaly, developmental delays, ataxia and seizures, which have been shown to worsen the ataxia.
Mitochondria Peroxisome Lysosome Unknown Unknown Unknown Unknown Unknown ASNS ADSL ASRGL1 ASL ASPA NARS ADSSL1 IL4I1 DARS ASS1 CAD NAT8L ABAT GAD1 DDO L-Aspartic acid Citrulline β-Alanine D-Aspartic acid D-Aspartic acid Oxalacetic acid L-Aspartic acid β-Alanine Oxalacetic acid Adenosine triphosphate L-Glutamine Water Adenosine monophosphate Pyrophosphate L-Asparagine L-Glutamic acid Fumaric acid Adenosine monophosphate Water Ammonia Argininosuccinic acid L-Arginine N-Acetyl-L- aspartic acid Water Acetic acid Adenosine triphosphate Adenosine monophosphate Pyrophosphate Guanosine triphosphate Inosinic acid Guanosine diphosphate Phosphate Adenylsuccinic acid Water Oxygen Ammonia Hydrogen peroxide Adenosine triphosphate Adenosine monophosphate Pyrophosphate Adenosine triphosphate Adenosine monophosphate Pyrophosphate Phosphate Ureidosuccinic acid Acetyl-CoA Coenzyme A Oxoglutaric acid Malonic semialdehyde L-Glutamic acid Carbon dioxide Water Oxygen Ammonia Hydrogen peroxide tRNA(Asn) L- Asparaginyl- tRNA(Asn) tRNA(Asp) L- Aspartyl- tRNA(Asp) Histidine Metabolism Urea Cycle Urea Cycle Arginine and Proline Metabolism beta-Alanine Metabolism Glycine and Serine Metabolism Nicotinate and Nicotinamide Metabolism Pyrimidine Metabolism Pantothenate and CoA Biosynthesis Oxalacetic acid Carbamoyl phosphate
ASNS ADSL ASRGL1 ASL ASPA NARS ADSSL1 IL4I1 DARS ASS1 CAD NAT8L ABAT GAD1 DDO Asp Citruln b-Ala D-Asp D-Asp Oaa Asp b-Ala Oaa ATP Gln H2O AMP PPi Asn Glu Fumarat AMP H2O NH3 ASA Arg NAcAsp H2O Acoh ATP AMP PPi GTP Imp GDP Pi Asuca H2O O2 NH3 H2O2 ATP AMP PPi ATP AMP PPi Pi USuccA Ac-CoA CoA AKG MalSah Glu CO2 H2O O2 NH3 H2O2 Zinc Mg2+ FAD Pyr-5'P FAD TRNAASN LATA TRNAASP LATA PW000043 PW000162 PW000162 PW000010 PW000011 PW000157 PW000151 PW000160 PW000032 Pyr-5'P Oaa CarbmP Zinc Intracellular Space Extracellular Space Mitochondrion Lysosome Peroxisome Symptoms of the disorder include microcephaly, developmental delays, ataxia and seizures, which have been shown to worsen the ataxia.
Mitochondria Peroxisome Lysosome ASNS ADSL ASRGL1 ASL ASPA NARS ADSSL1 IL4I1 DARS ASS1 CAD NAT8L ABAT GAD1 DDO Asp Citruln b-Ala D-Asp D-Asp Oaa Asp b-Ala Oaa ATP Gln H2O AMP Ppi Asn Glu Fumarat AMP H2O NH3 ASA Arg NAcAsp H2O Acoh ATP AMP Ppi GTP Imp GDP Pi Asuca H2O O2 NH3 H2O2 ATP AMP Ppi ATP AMP Ppi Pi USuccA Ac-CoA CoA AKG MalSah Glu CO2 H2O O2 NH3 H2O2 TRNAASN LATA TRNAASP LATA PW000043 PW000162 PW000162 PW000010 PW000011 PW000157 PW000151 PW000160 PW000032 Oaa CarbmP