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No. 薬物名（臨床試験情報から抽出） DrugBank KEGG DRUG KEGG GENES KEGG PATHWAY 指定難病告示番号
111C-ACETATE2件: Acetate,
Acetic acid C-11		---3件: 13, 86, 88
2[18F]Fluoro-2-deoxy-2-D-glucose1件: D-glucose1件: D00009 --2件: 86, 88
3Acetate1件: Acetate---43件: 1, 2, 6, 10, 13, 35, 40, 46, 49, 51, 60, 64, 67, 70, 71, 73, 75, 76, 78, 79, 81, 83, 85, 86, 88, 90, 96, 97, 113, 145, 156, 162, 171, 206, 222, 224, 225, 227, 235, 265, 288, 298, 299
4ACT-064992----5件: 51, 85, 86, 88, 210
5ACT-293987----3件: 51, 86, 88
6Adempas1件: Riociguat1件: D09572 3件: GUCY1A1,
GUCY1A2,
GUCY1B1 💬		11件: Circadian entrainment, Gap junction, Long-term depression, Metabolic pathways, Oxytocin signaling pathway, Platelet activation, Purine metabolism, Renin secretion, Salivary secretion, Vascular smooth muscle contraction, cGMP-PKG signaling pathway 💬5件: 51, 86, 88, 225, 299
7Adempas (active agent riociguat)1件: Riociguat1件: D09572 3件: GUCY1A1,
GUCY1A2,
GUCY1B1 💬		11件: Circadian entrainment, Gap junction, Long-term depression, Metabolic pathways, Oxytocin signaling pathway, Platelet activation, Purine metabolism, Renin secretion, Salivary secretion, Vascular smooth muscle contraction, cGMP-PKG signaling pathway 💬2件: 86, 88
8Adempas (Riociguat, BAY63-2521)1件: Riociguat1件: D09572 3件: GUCY1A1,
GUCY1A2,
GUCY1B1 💬		11件: Circadian entrainment, Gap junction, Long-term depression, Metabolic pathways, Oxytocin signaling pathway, Platelet activation, Purine metabolism, Renin secretion, Salivary secretion, Vascular smooth muscle contraction, cGMP-PKG signaling pathway 💬2件: 86, 88
9Adempas®1件: Riociguat1件: D09572 3件: GUCY1A1,
GUCY1A2,
GUCY1B1 💬		11件: Circadian entrainment, Gap junction, Long-term depression, Metabolic pathways, Oxytocin signaling pathway, Platelet activation, Purine metabolism, Renin secretion, Salivary secretion, Vascular smooth muscle contraction, cGMP-PKG signaling pathway 💬2件: 86, 88
10Ambrisentan1件: Ambrisentan1件: D07077 1件: EDNRA 💬7件: Calcium signaling pathway, Neuroactive ligand-receptor interaction, Pathways in cancer, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬7件: 51, 84, 85, 86, 88, 210, 211
11Ambrisentan 5 mg1件: Ambrisentan1件: D07077 1件: EDNRA 💬7件: Calcium signaling pathway, Neuroactive ligand-receptor interaction, Pathways in cancer, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬1件: 88
12Ammonia1件: Ammonia1件: D02916 --3件: 86, 88, 296
13Atorvastatin1件: Atorvastatin3件: D00258 ,
D00887 ,
D07474 		1件: HMGCR 💬4件: AMPK signaling pathway, Bile secretion, Metabolic pathways, Terpenoid backbone biosynthesis 💬18件: 13, 46, 49, 51, 58, 63, 79, 84, 88, 93, 96, 97, 160, 222, 225, 265, 271, 299
14Ballon Pulmonary Angioplasty----1件: 88
15Balloon Pulmonary Angioplasty (BPA)----1件: 88
16BARNASCAN----1件: 88
17BAY 63-2521----2件: 86, 88
18BAY 63-2521 coated tablets 0.5 mg----2件: 86, 88
19BAY 63-2521 coated tablets 1 mg----2件: 86, 88
20BAY 63-2521 coated tablets 1.5 mg----2件: 86, 88
21BAY 63-2521 coated tablets 2 mg----2件: 86, 88
22BAY 63-2521 coated tablets 2.5 mg----2件: 86, 88
23BAY 63-2521 IR coated tablet 1.0 mg----1件: 88
24BAY 63-2521 IR coated tablet 1.5 mg----1件: 88
25BAY 63-2521 IR coated tablet 2.0 mg----1件: 88
26BAY 63-2521 IR coated tablet 2.5 mg----1件: 88
27BAY 63-2521 IR tablet 0.5 mg----1件: 88
28BAY 63-2521 IR tablet 1.0 mg----1件: 88
29BAY 63-2521 IR tablet 1.5 mg----1件: 88
30BAY 63-2521 IR tablet 2.0 mg----1件: 88
31BAY 63-2521 IR tablet 2.5 mg----1件: 88
32BAY 63-2521 tablets 0.5 mg----2件: 86, 88
33BAY 63-2521 tablets 1 mg----2件: 86, 88
34BAY 63-2521 tablets 1.5 mg----2件: 86, 88
35BAY 63-2521 tablets 2 mg----2件: 86, 88
36BAY 63-2521 tablets 2.5 mg----2件: 86, 88
37BAY1237592----2件: 86, 88
38Behavioral: respiratory and exercise therapy----1件: 88
39Behavioral: respiratory and exercise therapy with supplemental oxygen1件: Oxygen1件: D00003 --1件: 88
40Bosentan1件: Bosentan2件: D01227 ,
D07538 		2件: EDNRA,
EDNRB 💬		9件: Calcium signaling pathway, Melanogenesis, Neuroactive ligand-receptor interaction, Pathways in cancer, Relaxin signaling pathway, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬11件: 13, 41, 47, 51, 84, 85, 86, 88, 210, 211, 212
41BOSENTAN MONOHYDRATE1件: Bosentan2件: D01227 ,
D07538 		2件: EDNRA,
EDNRB 💬		9件: Calcium signaling pathway, Melanogenesis, Neuroactive ligand-receptor interaction, Pathways in cancer, Relaxin signaling pathway, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬4件: 51, 84, 86, 88
42BPA----1件: 88
43Catheter directed therapy----1件: 88
44Cysteine1件: Cysteine2件: D00026 ,
D02326 		--11件: 6, 13, 19, 34, 84, 85, 88, 90, 254, 296, 310
45D-glucose1件: D-glucose1件: D00009 --3件: 49, 86, 88
46Diagnostic Test: Endogenous thrombin potential1件: Thrombin1件: D00090 --2件: 86, 88
47Diagnostic Test: Light transmission aggregometry----2件: 86, 88
48Diagnostic Test: Platelet function analyzer-100----2件: 86, 88
49Diagnostic Test: Rotational thromboelastometry----2件: 86, 88
50Edoxaban1件: Edoxaban1件: D09710 1件: F10 💬1件: Complement and coagulation cascades 💬2件: 46, 88
51Fludeoxyglucose1件: Fludeoxyglucose (18F)---4件: 34, 84, 85, 88
52Glucose1件: Dextrose, unspecified form---15件: 2, 6, 13, 49, 50, 65, 75, 86, 88, 96, 160, 195, 254, 257, 299
53GSK1325760----2件: 86, 88
54Imatinib1件: Imatinib2件: D01441 ,
D08066 		3件: ABL1,
KIT,
PDGFRA 💬		32件: Acute myeloid leukemia, Axon guidance, Breast cancer, Calcium signaling pathway, Cell cycle, Central carbon metabolism in cancer, Chemical carcinogenesis - reactive oxygen species, Choline metabolism in cancer, Chronic myeloid leukemia, EGFR tyrosine kinase inhibitor resistance, Endocytosis, ErbB signaling pathway, Focal adhesion, Gap junction, Glioma, Hematopoietic cell lineage, Human cytomegalovirus infection, JAK-STAT signaling pathway, MAPK signaling pathway, Melanogenesis, Melanoma, MicroRNAs in cancer, Neurotrophin signaling pathway, PI3K-Akt signaling pathway, Pathogenic Escherichia coli infection, Pathways in cancer, Phospholipase D signaling pathway, Prostate cancer, Rap1 signaling pathway, Ras signaling pathway, Regulation of actin cytoskeleton, Viral myocarditis 💬12件: 13, 28, 34, 35, 46, 51, 85, 86, 87, 88, 89, 162
55IMATINIB MESILATE1件: Imatinib2件: D01441 ,
D08066 		3件: ABL1,
KIT,
PDGFRA 💬		32件: Acute myeloid leukemia, Axon guidance, Breast cancer, Calcium signaling pathway, Cell cycle, Central carbon metabolism in cancer, Chemical carcinogenesis - reactive oxygen species, Choline metabolism in cancer, Chronic myeloid leukemia, EGFR tyrosine kinase inhibitor resistance, Endocytosis, ErbB signaling pathway, Focal adhesion, Gap junction, Glioma, Hematopoietic cell lineage, Human cytomegalovirus infection, JAK-STAT signaling pathway, MAPK signaling pathway, Melanogenesis, Melanoma, MicroRNAs in cancer, Neurotrophin signaling pathway, PI3K-Akt signaling pathway, Pathogenic Escherichia coli infection, Pathways in cancer, Phospholipase D signaling pathway, Prostate cancer, Rap1 signaling pathway, Ras signaling pathway, Regulation of actin cytoskeleton, Viral myocarditis 💬3件: 13, 86, 88
56Iodine1件: Iodine1件: D00108 --2件: 46, 88
57JNJ-67896062 / ACT-064992----2件: 86, 88
58JNJ-67896062/ ACT-064992----2件: 86, 88
59Long term oxygen therapy1件: Oxygen1件: D00003 --1件: 88
60Macitentan1件: Macitentan1件: D10135 2件: EDNRA,
EDNRB 💬		9件: Calcium signaling pathway, Melanogenesis, Neuroactive ligand-receptor interaction, Pathways in cancer, Relaxin signaling pathway, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬5件: 51, 85, 86, 88, 210
61Macitentan 10 mg1件: Macitentan1件: D10135 2件: EDNRA,
EDNRB 💬		9件: Calcium signaling pathway, Melanogenesis, Neuroactive ligand-receptor interaction, Pathways in cancer, Relaxin signaling pathway, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬3件: 51, 86, 88
62Macitentan 10mg1件: Macitentan1件: D10135 2件: EDNRA,
EDNRB 💬		9件: Calcium signaling pathway, Melanogenesis, Neuroactive ligand-receptor interaction, Pathways in cancer, Relaxin signaling pathway, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬2件: 51, 88
63MACITENTAN COMPRESSE1件: Macitentan1件: D10135 2件: EDNRA,
EDNRB 💬		9件: Calcium signaling pathway, Melanogenesis, Neuroactive ligand-receptor interaction, Pathways in cancer, Relaxin signaling pathway, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬2件: 86, 88
64MACITENTAN TABLETS1件: Macitentan1件: D10135 2件: EDNRA,
EDNRB 💬		9件: Calcium signaling pathway, Melanogenesis, Neuroactive ligand-receptor interaction, Pathways in cancer, Relaxin signaling pathway, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬2件: 86, 88
65Methyl 4,6-diamino-2-[1-(2-fluorobenzyl)-1H-pyrazolo [3,4-b]pyridine-3-yl]-5-pyrimidinyl(methyl)carb----2件: 86, 88
66Methyl [4,6-diamino-2-[1-[(2-fluorophenyl)methyl]-1H-pyrazolo[3,4-b]pyridin-3-yl]-5-pyrimidinyl]meth----2件: 86, 88
67MultiHance1件: Gadobenic acid2件: D04283 ,
D08018 		--2件: 86, 88
68N-acetyl Cysteine1件: Cysteine2件: D00026 ,
D02326 		--7件: 13, 19, 34, 85, 88, 296, 310
69NS-304----3件: 70, 86, 88
70NS-304 Placebo----2件: 70, 88
71Opsumit1件: Macitentan1件: D10135 2件: EDNRA,
EDNRB 💬		9件: Calcium signaling pathway, Melanogenesis, Neuroactive ligand-receptor interaction, Pathways in cancer, Relaxin signaling pathway, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬3件: 86, 88, 210
72Opsumit®1件: Macitentan1件: D10135 2件: EDNRA,
EDNRB 💬		9件: Calcium signaling pathway, Melanogenesis, Neuroactive ligand-receptor interaction, Pathways in cancer, Relaxin signaling pathway, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬3件: 86, 88, 210
73Oxygen1件: Oxygen1件: D00003 --20件: 6, 13, 19, 46, 49, 70, 84, 85, 86, 88, 90, 96, 97, 168, 193, 226, 271, 294, 299, 330
74Oxygen, liquid1件: Oxygen1件: D00003 --2件: 86, 88
75Placebo----27件: 3, 5, 6, 13, 14, 37, 40, 46, 49, 50, 51, 53, 63, 66, 67, 79, 85, 86, 88, 96, 97, 140, 144, 149, 226, 271, 276
76Potassium1件: Potassium1件: D08403 --12件: 36, 46, 66, 67, 88, 94, 98, 115, 167, 179, 215, 235
77Pulmonary endarterectomy----1件: 88
78Pulmonary Endarteriectomy----1件: 88
79Radiation: Cardiac PET imaging using F-18-FDG, N-13 ammonia( NH3)1件: Ammonia1件: D02916 --1件: 88
80Radiation: Computed tomography lung subtraction iodine mapping1件: Iodine1件: D00108 --1件: 88
81Remodulin1件: Treprostinil1件: D06213 1件: PTGIR 💬3件: Neuroactive ligand-receptor interaction, Platelet activation, Vascular smooth muscle contraction 💬2件: 86, 88
82Remodulin (R)1件: Treprostinil1件: D06213 1件: PTGIR 💬3件: Neuroactive ligand-receptor interaction, Platelet activation, Vascular smooth muscle contraction 💬1件: 88
83Remodulin 10mg/ml 20ml1件: Treprostinil1件: D06213 1件: PTGIR 💬3件: Neuroactive ligand-receptor interaction, Platelet activation, Vascular smooth muscle contraction 💬1件: 88
84Remodulin 1mg/ml 20ml1件: Treprostinil1件: D06213 1件: PTGIR 💬3件: Neuroactive ligand-receptor interaction, Platelet activation, Vascular smooth muscle contraction 💬1件: 88
85Remodulin 2.5mg/ml 20ml1件: Treprostinil1件: D06213 1件: PTGIR 💬3件: Neuroactive ligand-receptor interaction, Platelet activation, Vascular smooth muscle contraction 💬1件: 88
86Remodulin 5mg/ml 20ml1件: Treprostinil1件: D06213 1件: PTGIR 💬3件: Neuroactive ligand-receptor interaction, Platelet activation, Vascular smooth muscle contraction 💬1件: 88
87RIOCIGUAT1件: Riociguat1件: D09572 3件: GUCY1A1,
GUCY1A2,
GUCY1B1 💬		11件: Circadian entrainment, Gap junction, Long-term depression, Metabolic pathways, Oxytocin signaling pathway, Platelet activation, Purine metabolism, Renin secretion, Salivary secretion, Vascular smooth muscle contraction, cGMP-PKG signaling pathway 💬6件: 51, 84, 86, 88, 225, 299
88Riociguat (Adempas, BAY63-2521)1件: Riociguat1件: D09572 3件: GUCY1A1,
GUCY1A2,
GUCY1B1 💬		11件: Circadian entrainment, Gap junction, Long-term depression, Metabolic pathways, Oxytocin signaling pathway, Platelet activation, Purine metabolism, Renin secretion, Salivary secretion, Vascular smooth muscle contraction, cGMP-PKG signaling pathway 💬4件: 51, 86, 88, 299
89Riociguat Oral Tablet1件: Riociguat1件: D09572 3件: GUCY1A1,
GUCY1A2,
GUCY1B1 💬		11件: Circadian entrainment, Gap junction, Long-term depression, Metabolic pathways, Oxytocin signaling pathway, Platelet activation, Purine metabolism, Renin secretion, Salivary secretion, Vascular smooth muscle contraction, cGMP-PKG signaling pathway 💬1件: 88
90Ro 47-0203----4件: 51, 85, 86, 88
91Sauerstoff----2件: 86, 88
92Selexipag1件: Selexipag1件: D09994 1件: PTGIR 💬3件: Neuroactive ligand-receptor interaction, Platelet activation, Vascular smooth muscle contraction 💬6件: 47, 51, 70, 84, 86, 88
93Supplemental oxygen via a mask1件: Oxygen1件: D00003 --1件: 88
94Systemic anticoagulation----1件: 88
95Thrombin1件: Thrombin1件: D00090 --2件: 86, 88
96Tracleer1件: Bosentan2件: D01227 ,
D07538 		2件: EDNRA,
EDNRB 💬		9件: Calcium signaling pathway, Melanogenesis, Neuroactive ligand-receptor interaction, Pathways in cancer, Relaxin signaling pathway, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬8件: 13, 51, 84, 85, 86, 88, 210, 211
97TRACLEER 125MG 56CPR RIV.1件: Bosentan2件: D01227 ,
D07538 		2件: EDNRA,
EDNRB 💬		9件: Calcium signaling pathway, Melanogenesis, Neuroactive ligand-receptor interaction, Pathways in cancer, Relaxin signaling pathway, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬1件: 88
98TRACLEER*56CPR RIV 125MG1件: Bosentan2件: D01227 ,
D07538 		2件: EDNRA,
EDNRB 💬		9件: Calcium signaling pathway, Melanogenesis, Neuroactive ligand-receptor interaction, Pathways in cancer, Relaxin signaling pathway, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬3件: 51, 86, 88
99Treprostinil1件: Treprostinil1件: D06213 1件: PTGIR 💬3件: Neuroactive ligand-receptor interaction, Platelet activation, Vascular smooth muscle contraction 💬6件: 51, 84, 85, 86, 88, 210
100TREPROSTINIL SODIUM1件: Treprostinil1件: D06213 1件: PTGIR 💬3件: Neuroactive ligand-receptor interaction, Platelet activation, Vascular smooth muscle contraction 💬3件: 51, 86, 88
101Uptravi1件: Selexipag1件: D09994 1件: PTGIR 💬3件: Neuroactive ligand-receptor interaction, Platelet activation, Vascular smooth muscle contraction 💬3件: 84, 86, 88
102Volibris1件: Ambrisentan1件: D07077 1件: EDNRA 💬7件: Calcium signaling pathway, Neuroactive ligand-receptor interaction, Pathways in cancer, Renin secretion, Vascular smooth muscle contraction, cAMP signaling pathway, cGMP-PKG signaling pathway 💬4件: 51, 85, 86, 88
103Warfarin1件: Warfarin3件: D00564 ,
D01280 ,
D08682 		2件: NQO1,
VKORC1 💬		7件: Biosynthesis of cofactors, Chemical carcinogenesis - reactive oxygen species, Fluid shear stress and atherosclerosis, Hepatocellular carcinoma, Metabolic pathways, Pathways in cancer, Ubiquinone and other terpenoid-quinone biosynthesis 💬13件: 6, 8, 13, 19, 46, 49, 66, 85, 86, 88, 91, 96, 97
104Warfarin Potassium2件: Potassium,
Warfarin		4件: D00564 ,
D01280 ,
D08403 ,
D08682 		2件: NQO1,
VKORC1 💬		7件: Biosynthesis of cofactors, Chemical carcinogenesis - reactive oxygen species, Fluid shear stress and atherosclerosis, Hepatocellular carcinoma, Metabolic pathways, Pathways in cancer, Ubiquinone and other terpenoid-quinone biosynthesis 💬1件: 88