PMID-sentid	Pub_year	Sent_text	compound_name	comp_offset	prot_official_name	organism	prot_offset
33150193-0	2020	Two Siblings With Valproate-Related Hyperammonemia and Novel Mutations in Glutamine Synthetase (GLUL) Treated With Carglumic Acid.	carglumic acid	115-129	glutamate-ammonia ligase	Homo sapiens	74-94
33150193-0	2020	Two Siblings With Valproate-Related Hyperammonemia and Novel Mutations in Glutamine Synthetase (GLUL) Treated With Carglumic Acid.	carglumic acid	115-129	glutamate-ammonia ligase	Homo sapiens	96-100
31196016-0	2019	Evaluation of long-term effectiveness of the use of carglumic acid in patients with propionic acidemia (PA) or methylmalonic acidemia (MMA): study protocol for a randomized controlled trial.	carglumic acid	52-66	monocyte to macrophage differentiation associated	Homo sapiens	111-139
31196016-3	2019	Here we report the rationale and design of a phase IIIb trial that is aimed at determining the long-term efficacy and safety of carglumic acid in the management of PA and MMA.	carglumic acid	128-142	monocyte to macrophage differentiation associated	Homo sapiens	171-174
31196016-9	2019	Although retrospective studies have suggested that long-term carglumic acid may be beneficial in the management of PA and MMA, current literature lacks evidence for this indication.	carglumic acid	61-75	monocyte to macrophage differentiation associated	Homo sapiens	122-125
31196016-10	2019	This clinical trial will determine the long-term safety and efficacy of carglumic acid in the management of PA and MMA.	carglumic acid	72-86	monocyte to macrophage differentiation associated	Homo sapiens	115-118
30034506-1	2016	Introduction: N-carbamoyl-L-glutamic acid (NCG) is a synthetic analogue of N-acetyl glutamate (NAG) that works effectively as a cofactor for carbamoyl phosphate synthase 1 and enhances ureagenesis by activating the urea cycle.	carglumic acid	14-41	N-acetyl-alpha-glucosaminidase	Homo sapiens	75-93
30443462-4	2018	For these subtypes of patients, the United States Food and Drug Administration (US FDA) has recently approved carglumic acid, a medication that can act as a scavenger by effectively increasing the levels of NAGS, ultimately enhancing the conversion of ammonia to urea.	carglumic acid	110-124	N-acetylglutamate synthase	Homo sapiens	207-211
30034506-1	2016	Introduction: N-carbamoyl-L-glutamic acid (NCG) is a synthetic analogue of N-acetyl glutamate (NAG) that works effectively as a cofactor for carbamoyl phosphate synthase 1 and enhances ureagenesis by activating the urea cycle.	carglumic acid	14-41	N-acetyl-alpha-glucosaminidase	Homo sapiens	95-98
30034506-1	2016	Introduction: N-carbamoyl-L-glutamic acid (NCG) is a synthetic analogue of N-acetyl glutamate (NAG) that works effectively as a cofactor for carbamoyl phosphate synthase 1 and enhances ureagenesis by activating the urea cycle.	carglumic acid	14-41	carbamoyl-phosphate synthase 1	Homo sapiens	141-171
30034506-1	2016	Introduction: N-carbamoyl-L-glutamic acid (NCG) is a synthetic analogue of N-acetyl glutamate (NAG) that works effectively as a cofactor for carbamoyl phosphate synthase 1 and enhances ureagenesis by activating the urea cycle.	carglumic acid	43-46	N-acetyl-alpha-glucosaminidase	Homo sapiens	75-93
30034506-1	2016	Introduction: N-carbamoyl-L-glutamic acid (NCG) is a synthetic analogue of N-acetyl glutamate (NAG) that works effectively as a cofactor for carbamoyl phosphate synthase 1 and enhances ureagenesis by activating the urea cycle.	carglumic acid	43-46	N-acetyl-alpha-glucosaminidase	Homo sapiens	95-98
30034506-1	2016	Introduction: N-carbamoyl-L-glutamic acid (NCG) is a synthetic analogue of N-acetyl glutamate (NAG) that works effectively as a cofactor for carbamoyl phosphate synthase 1 and enhances ureagenesis by activating the urea cycle.	carglumic acid	43-46	carbamoyl-phosphate synthase 1	Homo sapiens	141-171
25302046-5	2014	The patient had a successful trial of carglumic acid (a synthetic analog of the product of nags), which suggests that, at least in some cases, he can be treated by overcoming proximal inhibition of the urea cycle.	carglumic acid	38-52	N-acetylglutamate synthase	Homo sapiens	91-95
30780843-3	2012	Carglumic acid (Carbaglu , Orphan Europe SARL, Paris, France) is approved for the acute and long-term treatment of primary defects of NAGS in Europe and the USA.	carglumic acid	0-14	N-acetylglutamate synthase	Homo sapiens	134-138
30780843-1	2012	Carglumic acid is a structural analog and the first registered synthetic form of the naturally occurring allosteric activator of the urea cycle, N-acetylglutamate (NAG), which is the product of the enzyme NAG synthase (NAGS).	carglumic acid	0-14	N-acetyl-alpha-glucosaminidase	Homo sapiens	164-167
30780843-3	2012	Carglumic acid (Carbaglu , Orphan Europe SARL, Paris, France) is approved for the acute and long-term treatment of primary defects of NAGS in Europe and the USA.	carglumic acid	16-24	N-acetylglutamate synthase	Homo sapiens	134-138
30780843-1	2012	Carglumic acid is a structural analog and the first registered synthetic form of the naturally occurring allosteric activator of the urea cycle, N-acetylglutamate (NAG), which is the product of the enzyme NAG synthase (NAGS).	carglumic acid	0-14	N-acetylglutamate synthase	Homo sapiens	205-217
30780843-1	2012	Carglumic acid is a structural analog and the first registered synthetic form of the naturally occurring allosteric activator of the urea cycle, N-acetylglutamate (NAG), which is the product of the enzyme NAG synthase (NAGS).	carglumic acid	0-14	N-acetylglutamate synthase	Homo sapiens	219-223
15050968-10	2004	A structural analog of NAG, carbamylglutamate, has been shown to bind and activate CPSI, and several patients have been reported to respond favorably to this drug (Carbaglu).	carglumic acid	164-172	N-acetyl-alpha-glucosaminidase	Homo sapiens	23-26
33763330-6	2021	We then suspected n-acetylglutamate synthase or carbamoyl-phosphate synthase I deficiency due to marked hyperammonemia, elevated glutamine level, normal orotic acid, and normalization of ammonia at 2 h of carglumic acid (200 mg/kg/d).	carglumic acid	205-219	N-acetylglutamate synthase	Homo sapiens	18-44