carbamoyl-phosphate synthase i deficiency disease; Carbamyl-Phosphate Synthetase I Deficiency Disease; Carbamoyl-Phosphate Synthase I Deficiency Disease (Ornithine Carbamoyl Phosphate Deficiency); CPS 1 Deficiency; Deficiency Disease, Carbamyl Phosphate Synthetase; Hyperammonemia Due to Carbamoyl Phosphate Synthetase 1 Deficiency

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Symptoms:

	Active principles	Rating	Bibliogr.
1	Carbamoyl-Phosphate Synthase (Ammonia)	100	+
2	Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing)	85	+
3	Complement C2	65	+
4	Benzoic Acid	37	+
5	Phosphorylase a	18	+
6	Dihydroorotase	18	+
7	Ornithine Carbamoyltransferase	16	+
8	Pyrimidine Nucleotides	12	+
9	Ligases	12	+
10	Ammonia	10	+
11	Phosphotransferases	9	+
12	Phenylacetates	9	+
13	Ornithine	8	+
14	Electron Transport Complex I	6	+
15	Phosphoribosyl Pyrophosphate	6	+
16	Phosphates	5	+
17	Carbamyl Phosphate	4	+
18	Ornithine Decarboxylase	3	+
19	Carboxyl and Carbamoyl Transferases	3	+
20	2',5'-Oligoadenylate Synthetase	3	+
21	NAD(P)H Dehydrogenase (Quinone)	3	+
22	Urea	2	+
23	GTP Cyclohydrolase	2	+
24	Glutamate-Ammonia Ligase	2	+
25	Acyl-CoA Dehydrogenases	2	+
26	NADH Dehydrogenase	2	+
27	Aspartate Carbamoyltransferase	2	+
28	Riboflavin	2	+
29	Carnitine	2	+
30	Quinone Reductases	2	+
31	NADH, NADPH Oxidoreductases	1	+
32	Amino-Acid N-Acetyltransferase	1	+
33	Thromboxane-A Synthase	1	+
34	Citrulline	1	+
35	Carbon-Nitrogen Ligases	1	+
36	Nitric Oxide Synthase	1	+
37	Argininosuccinate Synthase	1	+
38	Benzoates	1	+
39	Ornithine-Oxo-Acid Transaminase	1	+
40	Arginine	1	+
41	Iron	1	+
42	Biotin	1	+
43	Tetrahydronaphthalenes	1	+
44	Carbamates	1	+
45	Peptide Synthases	1	+

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