amino acid metabolism, inborn errors; Amino Acidopathies, Congenital

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amino acid metabolism, inborn errors; metabolism; metabolism, inborn errors

	Active principles	Rating	Bibliogr.
1	Amino Acids	100	+
2	Amino Acids, Branched-Chain	23	+
3	Amino Acids, Essential	22	+
4	Amino Acids, Sulfur	17	+
5	Leucine	14	+
6	Argininosuccinate Synthase	12	+
7	Alanine	11	+
8	Dietary Proteins	11	+
9	Isoleucine	10	+
10	Cystathionine	10	+
11	Methionine	9	+
12	Nitrogen	9	+
13	Proteins	9	+
14	Glutamine	9	+
15	Ornithine	9	+
16	Urea	9	+
17	Phenylalanine	8	+
18	Ketoglutaric Acids	8	+
19	Carbon Isotopes	8	+
20	Keto Acids	8	+
21	Glycine	8	+
22	Muscle Proteins	7	+
23	Glucose	7	+
24	Ammonia	7	+
25	Hydroxocobalamin	6	+
26	Carbamoyl-Phosphate Synthase (Ammonia)	6	+
27	Cysteine	6	+
28	Argininosuccinate Lyase	6	+
29	Excitatory Amino Acids	6	+
30	Serine	6	+
31	Pyridoxine	6	+
32	Carnitine	6	+
33	Deuterium	5	+
34	Insulin	5	+
35	Arginine	5	+
36	Glutamate-Ammonia Ligase	5	+
37	Lysine	5	+
38	Guanidinoacetate N-Methyltransferase	5	+
39	Cobamides	5	+
40	Benzoic Acid	5	+
41	Biogenic Monoamines	5	+
42	Amino Acid Transport Systems	5	+
43	Amino Acids, Aromatic	5	+
44	Blood Glucose	4	+
45	Threonine	4	+
46	Bile Acids and Salts	4	+
47	Arachidonic Acid	4	+
48	Carbohydrate Dehydrogenases	4	+
49	Glutarates	4	+
50	Amidinotransferases	4	+
51	Tryptophan	4	+
52	Propionates	4	+
53	Sarcosine	4	+
54	Receptors, Amino Acid	4	+
55	Methylmalonic Acid	3	+
56	Aspartic Acid	3	+
57	Amino Acids, Neutral	3	+
58	Histidine	3	+
59	Cholic Acid	3	+
60	Fatty Acids	3	+
61	Amino Acids, Cyclic	3	+
62	Hippurates	3	+
63	Phenylacetates	3	+
64	Caseins	3	+
65	Methyltransferases	3	+
66	Arachidonic Acids	3	+
67	Ligases	3	+
68	Cystathionine beta-Synthase	3	+
69	Taurine	3	+
70	Phosphoglycerate Dehydrogenase	3	+
71	Carbon Radioisotopes	3	+
72	Glutamic Acid	3	+
73	Flavoproteins	3	+
74	Iron-Sulfur Proteins	3	+
75	Tyrosine	2	+
76	Pentanoic Acids	2	+
77	Creatine	2	+
78	Phenylalanine Hydroxylase	2	+
79	Multienzyme Complexes	2	+
80	Cystine	2	+
81	Amino Acid Transport Systems, Basic	2	+
82	Nucleocytoplasmic Transport Proteins	2	+
83	Electron-Transferring Flavoproteins	2	+
84	Phenylbutyrates	2	+
85	Fatty Acids, Nonesterified	2	+
86	Vitamin B 12	2	+
87	Biopterin	2	+
88	Biotinidase	2	+
89	Cholic Acids	2	+
90	Fatty Acid Desaturases	2	+
91	Dietary Fats	2	+
92	Biotin	2	+
93	Nitrogen Isotopes	2	+
94	Codon, Nonsense	2	+
95	Acyl-CoA Dehydrogenase	2	+
96	Oxidoreductases	2	+
97	Oxidoreductases Acting on CH-CH Group Donors	2	+
98	Valine	2	+
99	Oxidoreductases Acting on CH-NH Group Donors	2	+
100	Large Neutral Amino Acid-Transporter 1	2	+
101	Milk Proteins	2	+
102	Amino Acid Transport Systems, Neutral	2	+
103	Amino Acids, Acidic	2	+
104	Leukotriene B4	2	+
105	Lipoxygenase	2	+
106	Protein Hydrolysates	1	+
107	Triglycerides	1	+
108	Aminoisobutyric Acids	1	+
109	Phospholipases A	1	+
110	Dipeptides	1	+
111	Cholesterol	1	+
112	Peptides	1	+
113	Linoleoyl-CoA Desaturase	1	+
114	Glucagon	1	+
115	Acyl-CoA Dehydrogenases	1	+
116	Blood Proteins	1	+
117	Benzoates	1	+
118	Ketone Bodies	1	+
119	Asparagine	1	+
120	Lipids	1	+
121	Cytochrome P-450 Enzyme System	1	+
122	Amino Acids, Basic	1	+
123	Dietary Carbohydrates	1	+
124	Phospholipids	1	+
125	Fatty Acids, Unsaturated	1	+
126	Amino Acids, Diamino	1	+
127	Methylhistidines	1	+
128	Tritium	1	+
129	RNA, Transfer, Amino Acyl	1	+
130	Fatty Acids, Essential	1	+
131	Phospholipases A2	1	+
132	Palmitates	1	+
133	Amino Acid Transport System ASC	1	+
134	Uric Acid	1	+
135	Carbon Dioxide	1	+
136	Palmitic Acid	1	+
137	Lithocholic Acid	1	+
138	12-Hydroxy-5,8,10,14-eicosatetraenoic Acid	1	+
139	Eicosanoids	1	+
140	Palmitic Acids	1	+
141	Calcimycin	1	+
142	Citrulline	1	+
143	Iodobenzenes	1	+
144	Acyl Coenzyme A	1	+
145	Excitatory Amino Acid Transporter 1	1	+
146	Hydroxyeicosatetraenoic Acids	1	+
147	Electrolytes	1	+
148	Carbohydrates	1	+
149	Sulfur	1	+
150	RNA, Messenger	1	+
151	Glutamates	1	+
152	Calcium	1	+
153	Prostaglandin-Endoperoxide Synthases	1	+
154	Peptide Fragments	1	+
155	Carboxy-Lyases	1	+
156	5,8,11,14-Eicosatetraynoic Acid	1	+
157	4,5-Dihydro-1-(3-(trifluoromethyl)phenyl)-1H-pyrazol-3-amine	1	+
158	Linoleic Acid	1	+
159	Excitatory Amino Acid Transporter 3	1	+
160	Soybean Proteins	1	+
161	Coenzyme A	1	+
162	N-Methylaspartate	1	+
163	Lactalbumin	1	+
164	Amino Alcohols	1	+
165	Dinoprostone	1	+
166	Amino Acids, Dicarboxylic	1	+
167	Quisqualic Acid	1	+
168	Serum Albumin	1	+
169	Deoxycholic Acid	1	+
170	Lactates	1	+
171	Carnitine O-Palmitoyltransferase	1	+
172	Arachidonate 5-Lipoxygenase	1	+
173	Carrier Proteins	1	+
174	Glycerol	1	+
175	Masoprocol	1	+
176	alpha-Linolenic Acid	1	+
177	8,11,14-Eicosatrienoic Acid	1	+
178	Malonates	1	+
179	DNA, Complementary	1	+
180	Amino Acid Transport System X-AG	1	+
181	Sulfates	1	+
182	Plant Oils	1	+
183	gamma-Aminobutyric Acid	1	+
184	Proteome	1	+
185	Ethanol	1	+
186	Chenodeoxycholic Acid	1	+
187	Serotonin	1	+
188	Plant Proteins	1	+
189	Prostaglandins	1	+
190	Fish Proteins	1	+
191	Hydroxyindoleacetic Acid	1	+
192	Homoarginine	1	+
193	Norleucine	1	+
194	Kynurenic Acid	1	+
195	PPAR alpha	1	+
196	Glutathione	1	+
197	Prealbumin	1	+
198	Ethers, Cyclic	1	+
199	Palmitoylcarnitine	1	+
200	gamma-Linolenic Acid	1	+
201	Adenylate Kinase	1	+
202	Creatinine	1	+
203	Sterols	1	+
204	Thromboxanes	1	+
205	Linoleic Acids	1	+
206	Indomethacin	1	+
207	Vegetable Proteins	1	+
208	RNA	1	+
209	Malonyl Coenzyme A	1	+
210	Fish Oils	1	+

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