Treatment of hyperammonaemia due to N-acetylglutamate synthase (NAGS) deficiency
Target
CPS1 [HSA:1373] [KO:K01948]
Network
N10011
CPS1 activator for deficient NAGS
Pathway
hsa00220
Arginine biosynthesis
hsa00250
Alanine, aspartate and glutamate metabolism
hsa00910
Nitrogen metabolism
Brite
Anatomical Therapeutic Chemical (ATC) classification [BR:br08303]
A ALIMENTARY TRACT AND METABOLISM
A16 OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS
A16A OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS
A16AA Amino acids and derivatives
A16AA05 Carglumic acid
D07130 Carglumic acid (JAN/USAN/INN) <JP/US>
USP drug classification [BR:br08302]
Electrolytes/Minerals/Metals/Vitamins
Electrolyte/Mineral Replacement
Carglumic Acid
D07130 Carglumic acid (JAN/USAN/INN)
Therapeutic category of drugs in Japan [BR:br08301]
3 Agents affecting metabolism
39 Other agents affecting metabolism
399 Miscellaneous
3999 Others
D07130 Carglumic acid (JAN/USAN/INN)
Target-based classification of drugs [BR:br08310]
Enzymes
Ligases (EC6)
Carbamoyl-phosphate synthase
CPS1
D07130 Carglumic acid (JAN/USAN/INN) <JP/US>
New drug approvals in the USA [br08319.html]
New molecular entities and new therapeutic biological products
D07130
New drug approvals in Europe [br08329.html]
European public assessment reports (EPAR) authorised medicine
D07130
New drug approvals in Japan [br08318.html]
Drugs with new active ingredients
D07130
New drug approvals in the USA, Europe and Japan [br08328.html]
Approval dates by FDA, EMA and PMDA
D07130
Pharmacogenomic biomarkers [br08341.html]
Germline mutations in genetic disorder treatments
D07130
Other DBs
CAS:
1188-38-1
PubChem:
51091469
ChEBI:
71028
LigandBox:
D07130
KCF data
ATOM 13
1 O6a O 14.9813 -16.8715
2 C6a C 16.1938 -17.5716
3 C1b C 17.4064 -16.8715
4 C1b C 18.6189 -17.5716
5 C1c C 19.8314 -16.8715
6 C6a C 21.0440 -17.5716
7 O6a O 22.2565 -16.8715
8 O6a O 16.1938 -18.9715
9 O6a O 21.0440 -18.9714
10 N1b N 19.8314 -15.4715
11 C5a C 18.6000 -14.7699
12 O5a O 18.6017 -13.3698
13 N1a N 17.4047 -15.4638
BOND 12
1 1 2 1
2 2 3 1
3 3 4 1
4 4 5 1
5 5 6 1
6 6 7 1
7 2 8 2
8 6 9 2
9 5 10 1 #Down
10 10 11 1
11 11 12 2
12 11 13 1