Sapropterin dihydrochloride (BioDeep_00000708765)
代谢物信息卡片
(6R)-5,6,7,8-Tetrahydro-L-biopterin dihydrochloride
化学式: C9H17Cl2N5O3 (313.0708392)
中文名称: (6R)-5678-四氢生物蝶呤二盐酸盐
谱图信息:
最多检出来源 () 0%
分子结构信息
SMILES: CC(C(C1CNC2=C(N1)C(=O)NC(=N2)N)O)O.Cl.Cl
InChI: /m0../s1
描述信息
C26170 - Protective Agent > C275 - Antioxidant
同义名列表
数据库引用编号
6 个数据库交叉引用编号
- ChEBI: CHEBI:32120
- KEGGdrug: D82236
- KEGGdrug: D01798
- PubChem: 135409471
- ChEMBL: CHEMBL1201775
- CAS: 69056-38-8
分类词条
相关代谢途径
Reactome(0)
BioCyc(0)
PlantCyc(0)
代谢反应
0 个相关的代谢反应过程信息。
Reactome(0)
BioCyc(0)
WikiPathways(0)
Plant Reactome(0)
INOH(0)
PlantCyc(0)
COVID-19 Disease Map(0)
PathBank(0)
PharmGKB(0)
0 个相关的物种来源信息
在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有：
- PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
- NCBI Taxonomy: 通过文献数据挖掘，得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序，取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
- Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
- Chemical Reaction: 在化学反应过程中，存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。
点击图上的相关代谢物的名称，可以跳转到相关代谢物的信息页面。
文献列表
- Katherine A Kelly, Cristine L Heaps, Guoyao Wu, Vinod Labhasetwar, Cynthia J Meininger. Nanoparticle-mediated delivery of tetrahydrobiopterin restores endothelial function in diabetic rats.
Nitric oxide : biology and chemistry.
2024 Jul; 148(?):13-22. doi:
10.1016/j.niox.2024.04.009. [PMID: 38642795] - Zengyi Liu, Ruixin Kang, Ning Yang, Xiuhua Pan, Jie Yang, Hongjie Yu, Wanli Deng, Zengguang Jia, Jun Zhang, Qi Shen. Tetrahydrobiopterin inhibitor-based antioxidant metabolic strategy for enhanced cancer ferroptosis-immunotherapy.
Journal of colloid and interface science.
2024 Mar; 658(?):100-113. doi:
10.1016/j.jcis.2023.12.042. [PMID: 38100967] - Zijing Wang, Nan Zhang, Miao Zhang, Yao Jiang, Aik Seng Ng, Esther Bridges, Wei Zhang, Xin Zeng, Qi Luo, Jiabien Liang, Balázs Győrffy, Philip Hublitz, Zhu Liang, Roman Fischer, David Kerr, Adrian L Harris, Shijie Cai. GTP Cyclohydrolase Drives Breast Cancer Development and Promotes EMT in an Enzyme-Independent Manner.
Cancer research.
2023 Oct; 83(20):3400-3413. doi:
10.1158/0008-5472.can-22-3471. [PMID: 37463466] - Long-Ji Ze, Ping Xu, Jian-Jian Wu, Lin Jin, Ahmad Ali Anjum, Guo-Qing Li. Disruption of tetrahydrobiopterin (BH4) biosynthesis pathway affects cuticle pigmentation in Henosepilachna vigintioctopunctata.
Journal of insect physiology.
2023 01; 144(?):104457. doi:
10.1016/j.jinsphys.2022.104457. [PMID: 36427533] - Zongtao Liu, Nianguo Dong, Haipeng Hui, Yixuan Wang, Fayun Liu, Li Xu, Ming Liu, Zhenqi Rao, Zhen Yuan, Yuqiang Shang, Jun Feng, Zhejun Cai, Fei Li. Endothelial cell-derived tetrahydrobiopterin prevents aortic valve calcification.
European heart journal.
2022 05; 43(17):1652-1664. doi:
10.1093/eurheartj/ehac037. [PMID: 35139535] - Katsuya Miyajima, Yusuke Sudo, Sho Sanechika, Yoshitaka Hara, Mieko Horiguchi, Feng Xu, Minori Suzuki, Satoshi Hara, Koichi Tanda, Ken-Ichi Inoue, Masahiko Takada, Nozomu Yoshioka, Hirohide Takebayashi, Masayo Mori-Kojima, Masahiro Sugimoto, Chiho Sumi-Ichinose, Kazunao Kondo, Keizo Takao, Tsuyoshi Miyakawa, Hiroshi Ichinose. Perturbation of monoamine metabolism and enhanced fear responses in mice defective in the regeneration of tetrahydrobiopterin.
Journal of neurochemistry.
2022 04; 161(2):129-145. doi:
10.1111/jnc.15600. [PMID: 35233765] - Angela V Bisconti, Ryan S Garten, Ryan M Broxterman, Catherine L Jarrett, Soung Hun Park, Katherine L Shields, Heather L Clifton, Stephen M Ratchford, Van Reese, Jia Zhao, D Walter Wray, Russell S Richardson. No effect of acute tetrahydrobiopterin (BH4) supplementation on vascular dysfunction in the old.
Journal of applied physiology (Bethesda, Md. : 1985).
2022 03; 132(3):773-784. doi:
10.1152/japplphysiol.00711.2021. [PMID: 35112931] - Atanu Sen, Archna Singh, Ambuj Roy, Sujata Mohanty, Nitish Naik, Mani Kalaivani, Lakshmy Ramakrishnan. Role of endothelial colony forming cells (ECFCs) Tetrahydrobiopterin (BH4) in determining ECFCs functionality in coronary artery disease (CAD) patients.
Scientific reports.
2022 02; 12(1):3076. doi:
10.1038/s41598-022-06758-8. [PMID: 35197509] - Mina Tamura, Shizuka Seki, Yasuyuki Kakurai, Shuichi Chikada, Kento Wada. Sapropterin for phenylketonuria: A Japanese post-marketing surveillance study.
Pediatrics international : official journal of the Japan Pediatric Society.
2022 Jan; 64(1):e14939. doi:
10.1111/ped.14939. [PMID: 34331785] - Kathrin M Engel, Sven Baumann, Janet Blaurock, Ulrike Rolle-Kampczyk, Jürgen Schiller, Martin von Bergen, Sonja Grunewald. Differences in the sperm metabolomes of smoking and nonsmoking men†.
Biology of reproduction.
2021 12; 105(6):1484-1493. doi:
10.1093/biolre/ioab179. [PMID: 34554205] - Muriel Bouly, Marie-Pierre Bourguignon, Susanne Roesch, Pascal Rigouin, Willy Gosgnach, Elodie Bossard, Emilie Royere, Nicolas Diguet, Patricia Sansilvestri-Morel, Ariane Bonnin, Laura Xuereb, Pascal Berson, Michel Komajda, Peter Bernhardt, Benoit Tyl. Aging increases circulating BH2 without modifying BH4 levels and impairs peripheral vascular function in healthy adults.
Translational research : the journal of laboratory and clinical medicine.
2021 12; 238(?):36-48. doi:
10.1016/j.trsl.2021.07.004. [PMID: 34332154] - Gabor Czibik, Zaineb Mezdari, Dogus Murat Altintas, Juliette Bréhat, Maria Pini, Thomas d'Humières, Thaïs Delmont, Costin Radu, Marielle Breau, Hao Liang, Cecile Martel, Azania Abatan, Rizwan Sarwar, Ophélie Marion, Suzain Naushad, Yanyan Zhang, Maissa Halfaoui, Nadine Suffee, Didier Morin, Serge Adnot, Stéphane Hatem, Arash Yavari, Daigo Sawaki, Geneviève Derumeaux. Dysregulated Phenylalanine Catabolism Plays a Key Role in the Trajectory of Cardiac Aging.
Circulation.
2021 08; 144(7):559-574. doi:
10.1161/circulationaha.121.054204. [PMID: 34162223] - Katja Schmitz, Sandra Trautmann, Lisa Hahnefeld, Caroline Fischer, Yannick Schreiber, Annett Wilken-Schmitz, Robert Gurke, Robert Brunkhorst, Ernst R Werner, Katrin Watschinger, Sabine Wicker, Dominique Thomas, Gerd Geisslinger, Irmgard Tegeder. Sapropterin (BH4) Aggravates Autoimmune Encephalomyelitis in Mice.
Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics.
2021 07; 18(3):1862-1879. doi:
10.1007/s13311-021-01043-4. [PMID: 33844153] - Shelly A Buffington, Sean W Dooling, Martina Sgritta, Cecilia Noecker, Oscar D Murillo, Daniela F Felice, Peter J Turnbaugh, Mauro Costa-Mattioli. Dissecting the contribution of host genetics and the microbiome in complex behaviors.
Cell.
2021 04; 184(7):1740-1756.e16. doi:
10.1016/j.cell.2021.02.009. [PMID: 33705688] - Ricardo Carnicer, Drew Duglan, Klemen Ziberna, Alice Recalde, Svetlana Reilly, Jillian N Simon, Simona Mafrici, Ritu Arya, Esther Roselló-Lletí, Surawee Chuaiphichai, Damian Tyler, Craig A Lygate, Keith M Channon, Barbara Casadei. BH4 Increases nNOS Activity and Preserves Left Ventricular Function in Diabetes.
Circulation research.
2021 03; 128(5):585-601. doi:
10.1161/circresaha.120.316656. [PMID: 33494625] - Mariluz Soula, Ross A Weber, Omkar Zilka, Hanan Alwaseem, Konnor La, Frederick Yen, Henrik Molina, Javier Garcia-Bermudez, Derek A Pratt, Kıvanç Birsoy. Metabolic determinants of cancer cell sensitivity to canonical ferroptosis inducers.
Nature chemical biology.
2020 12; 16(12):1351-1360. doi:
10.1038/s41589-020-0613-y. [PMID: 32778843] - Helene Nehring, Svenja Meierjohann, Jose Pedro Friedmann Angeli. Emerging aspects in the regulation of ferroptosis.
Biochemical Society transactions.
2020 10; 48(5):2253-2259. doi:
10.1042/bst20200523. [PMID: 33125483] - Ronja Hesthammer, Stian Dahle, Jon Peder Storesund, Torunn Eide, Rune Djurhuus, Asbjørn M Svardal, Einar Thorsen. Nitric oxide in exhaled gas and tetrahydrobiopterin in plasma after exposure to hyperoxia.
Undersea & hyperbaric medicine : journal of the Undersea and Hyperbaric Medical Society, Inc.
2020 Sec; 47(2):197-202. doi:
. [PMID: 32574435] - Masahide Fujita, Débora da Luz Scheffer, Bruna Lenfers Turnes, Shane J F Cronin, Alban Latrémolière, Michael Costigan, Clifford J Woolf, Alexandra Latini, Nick A Andrews. Sepiapterin Reductase Inhibition Leading to Selective Reduction of Inflammatory Joint Pain in Mice and Increased Urinary Sepiapterin Levels in Humans and Mice.
Arthritis & rheumatology (Hoboken, N.J.).
2020 01; 72(1):57-66. doi:
10.1002/art.41060. [PMID: 31350812] - James T Meyer, Brian A Sparling, William J McCarty, Maosheng Zhang, Marcus Soto, Stephen Schneider, Hao Chen, Jonathan Roberts, Helming Tan, Thomas Kornecook, Paul S Andrews, Charles G Knutson. Pharmacological Assessment of Sepiapterin Reductase Inhibition on Tactile Response in the Rat.
The Journal of pharmacology and experimental therapeutics.
2019 11; 371(2):476-486. doi:
10.1124/jpet.119.257105. [PMID: 31110114] - Juan Carlos Torres-Narváez, Israel Pérez-Torres, Vicente Castrejón-Téllez, Elvira Varela-López, Víctor Hugo Oidor-Chan, Verónica Guarner-Lans, Álvaro Vargas-González, Raúl Martínez-Memije, Pedro Flores-Chávez, Etzna Zizith Cervantes-Yañez, Claudia Angélica Soto-Peredo, Gustavo Pastelín-Hernández, Leonardo Del Valle-Mondragón. The Role of the Activation of the TRPV1 Receptor and of Nitric Oxide in Changes in Endothelial and Cardiac Function and Biomarker Levels in Hypertensive Rats.
International journal of environmental research and public health.
2019 09; 16(19):. doi:
10.3390/ijerph16193576. [PMID: 31557799] - Peyman Eshraghi, Samaneh Noroozi Asl, Sepideh Bagheri, Vajiheh Chalak. Response to sapropterin hydrochloride (Kuvan®) in children with phenylketonuria (PKU): a clinical trial.
Journal of pediatric endocrinology & metabolism : JPEM.
2019 Aug; 32(8):885-888. doi:
10.1515/jpem-2018-0503. [PMID: 31237861] - Neil Smith, Nicola Longo, Keith Levert, Keith Hyland, Nenad Blau. Phase I clinical evaluation of CNSA-001 (sepiapterin), a novel pharmacological treatment for phenylketonuria and tetrahydrobiopterin deficiencies, in healthy volunteers.
Molecular genetics and metabolism.
2019 04; 126(4):406-412. doi:
10.1016/j.ymgme.2019.02.001. [PMID: 30922814] - M Kuczeriszka, A Walkowska, K H Olszynski, J Rafalowska, J Sadowski, E Kompanowska-Jezierska. Arginine and tetrahydrobiopterin supplementation in rats with salt-induced blood pressure increase: minor hypotensive effect but improvement of renal haemodynamics.
Journal of physiology and pharmacology : an official journal of the Polish Physiological Society.
2019 Apr; 70(2):. doi:
10.26402/jpp.2019.2.05. [PMID: 31356183] - Sibtain Ahmed, Hafsa Majid, Lena Jafri, Aysha Habib Khan, Zeba Zulfiqar Ali, Bushra Afroze. Retrospective Study of Patients with Hyperphenylalaninemia- Experience from a Tertiary Care Center in Pakistan.
JPMA. The Journal of the Pakistan Medical Association.
2019 04; 69(4):509-513. doi:
NULL. [PMID: 31000854] - Katja Zschiebsch, Caroline Fischer, Annett Wilken-Schmitz, Gerd Geisslinger, Keith Channon, Katrin Watschinger, Irmgard Tegeder. Mast cell tetrahydrobiopterin contributes to itch in mice.
Journal of cellular and molecular medicine.
2019 02; 23(2):985-1000. doi:
10.1111/jcmm.13999. [PMID: 30450838] - Marwan Al-Nimer, Rawa Ratha, Taha Mahwi. Utility of Tetrahydrobiopterin Pathway in the Assessment of Diabetic Foot Ulcer: Significant and Complex Interrelations.
Journal of diabetes research.
2019; 2019(?):3426878. doi:
10.1155/2019/3426878. [PMID: 31828160] - Jaume Campistol Plana. [Early diagnosis of phenylketonuria. Physiopathology of the neuronal damage and therapeutic options].
Medicina.
2019; 79 Suppl 3(?):2-5. doi:
NULL. [PMID: 31603834] - Jin Hee Jeong, Nichole Lee, Matthew A Tucker, Paula Rodriguez-Miguelez, Jacob Looney, Jeffrey Thomas, Casandra C Derella, Ahmed El-Marakby, Jacqueline B Musall, Jennifer C Sullivan, Kathleen T McKie, Caralee Forseen, Gareth W Davison, Ryan A Harris. Tetrahydrobiopterin improves endothelial function in patients with cystic fibrosis.
Journal of applied physiology (Bethesda, Md. : 1985).
2019 01; 126(1):60-66. doi:
10.1152/japplphysiol.00629.2018. [PMID: 30433862] - Dalia G Mostafa, Salwa Fares Ahmed, Ola A Hussein. Protective effect of tetrahydrobiopterin on hepatic and renal damage after acute cadmium exposure in male rats.
Ultrastructural pathology.
2018 Nov; 42(6):516-531. doi:
10.1080/01913123.2018.1559566. [PMID: 30595070] - Kristen D Brantley, Teresa D Douglas, Rani H Singh. One-year follow-up of B vitamin and Iron status in patients with phenylketonuria provided tetrahydrobiopterin (BH4).
Orphanet journal of rare diseases.
2018 10; 13(1):192. doi:
10.1186/s13023-018-0923-2. [PMID: 30373601] - Zhiqi Tang, Lijuan Liu, Yujie Guo, Guoxiong Deng, Meixiang Chen, Jinru Wei. Exendin‑4 reverses endothelial dysfunction in mice fed a high‑cholesterol diet by a GTP cyclohydrolase‑1/tetrahydrobiopterin pathway.
Molecular medicine reports.
2018 Sep; 18(3):3350-3358. doi:
10.3892/mmr.2018.9345. [PMID: 30085331] - Paula Rodriguez-Miguelez, Justin Gregg, Nichole Seigler, Leon Bass, Jeffrey Thomas, Jennifer S Pollock, Jennifer C Sullivan, Thomas A Dillard, Ryan A Harris. Acute Tetrahydrobiopterin Improves Endothelial Function in Patients With COPD.
Chest.
2018 09; 154(3):597-606. doi:
10.1016/j.chest.2018.04.028. [PMID: 29705218] - Arafat Nasser, Anette Torvin Møller, Vibe Hellmund, Sidsel Salling Thorborg, Cathrine Jespersgaard, Ole J Bjerrum, Erik Dupont, Gösta Nachman, Jens Lykkesfeldt, Troels Staehelin Jensen, Lisbeth Birk Møller. Heterozygous mutations in GTP-cyclohydrolase-1 reduce BH4 biosynthesis but not pain sensitivity.
Pain.
2018 Jun; 159(6):1012-1024. doi:
10.1097/j.pain.0000000000001175. [PMID: 29470312] - Jinqiu Wei, Yujiao Zhang, Zhan Li, Ximin Wang, Linlin Chen, Juanjuan Du, Jing Liu, Ju Liu, Yinglong Hou. GCH1 attenuates cardiac autonomic nervous remodeling in canines with atrial-tachypacing via tetrahydrobiopterin pathway regulated by microRNA-206.
Pacing and clinical electrophysiology : PACE.
2018 05; 41(5):459-471. doi:
10.1111/pace.13289. [PMID: 29436714] - James D Clelland, Laura L Read, Jennifer Smeed, Catherine L Clelland. Regulation of cortical and peripheral GCH1 expression and biopterin levels in schizophrenia-spectrum disorders.
Psychiatry research.
2018 04; 262(?):229-236. doi:
10.1016/j.psychres.2018.02.020. [PMID: 29471261] - Cristina Espinosa-Díez, Verónica Miguel, Susana Vallejo, Francisco J Sánchez, Elena Sandoval, Eva Blanco, Pablo Cannata, Concepción Peiró, Carlos F Sánchez-Ferrer, Santiago Lamas. Role of glutathione biosynthesis in endothelial dysfunction and fibrosis.
Redox biology.
2018 04; 14(?):88-99. doi:
10.1016/j.redox.2017.08.019. [PMID: 28888203] - Teng-Fei Yuan, Han-Qi Huang, Ling Gao, Shao-Ting Wang, Yan Li. A novel and reliable method for tetrahydrobiopterin quantification: Benzoyl chloride derivatization coupled with liquid chromatography-tandem mass spectrometry analysis.
Free radical biology & medicine.
2018 04; 118(?):119-125. doi:
10.1016/j.freeradbiomed.2018.02.035. [PMID: 29501564] - Hiroyuki Kinoshita, Kazunobu Otake, Takashi Yamasaki. The Unknown Mechanism of Exogenous Tetrahydrobiopterin in the Renal Protection of Sheep Ischemia and Reperfusion.
Anesthesia and analgesia.
2018 03; 126(3):1088. doi:
10.1213/ane.0000000000002784. [PMID: 29346125] - Akiko Ohashi, Kaori Mamada, Tomonori Harada, Masako Naito, Tomihisa Takahashi, Shin Aizawa, Hiroyuki Hasegawa. Organic anion transporters, OAT1 and OAT3, are crucial biopterin transporters involved in bodily distribution of tetrahydrobiopterin and exclusion of its excess.
Molecular and cellular biochemistry.
2017 Nov; 435(1-2):97-108. doi:
10.1007/s11010-017-3060-7. [PMID: 28534121] - Simone R Potje, Zhenlong Chen, Suellen D'Arc S Oliveira, Lusiane M Bendhack, Roberto S da Silva, Marcelo G Bonini, Cristina Antoniali, Richard D Minshall. Nitric oxide donor [Ru(terpy)(bdq)NO]3+ induces uncoupling and phosphorylation of endothelial nitric oxide synthase promoting oxidant production.
Free radical biology & medicine.
2017 11; 112(?):587-596. doi:
10.1016/j.freeradbiomed.2017.09.004. [PMID: 28899725] - Jiao Xue, Chenxiao Yu, Wenjiong Sheng, Wei Zhu, Judong Luo, Qi Zhang, Hongying Yang, Han Cao, Wenjie Wang, Jundong Zhou, Jinchang Wu, Peng Cao, Ming Chen, Wei-Qun Ding, Jianping Cao, Shuyu Zhang. The Nrf2/GCH1/BH4 Axis Ameliorates Radiation-Induced Skin Injury by Modulating the ROS Cascade.
The Journal of investigative dermatology.
2017 10; 137(10):2059-2068. doi:
10.1016/j.jid.2017.05.019. [PMID: 28596000] - Lokmane Rahmania, Diego Orbegozo, Fuhong Su, Fabio Silvio Taccone, Jean-Louis Vincent, Daniel De Backer. Administration of Tetrahydrobiopterin (BH4) Protects the Renal Microcirculation From Ischemia and Reperfusion Injury.
Anesthesia and analgesia.
2017 10; 125(4):1253-1260. doi:
10.1213/ane.0000000000002131. [PMID: 28632534] - Gry Freja Skovsted, Pernille Tveden-Nyborg, Maiken Marie Lindblad, Stine Normann Hansen, Jens Lykkesfeldt. Vitamin C Deficiency Reduces Muscarinic Receptor Coronary Artery Vasoconstriction and Plasma Tetrahydrobiopterin Concentration in Guinea Pigs.
Nutrients.
2017 Jul; 9(7):. doi:
10.3390/nu9070691. [PMID: 28671625] - Francesca Nardecchia, Flavia Chiarotti, Claudia Carducci, Silvia Santagata, Giulia Valentini, Antonio Angeloni, Nenad Blau, Vincenzo Leuzzi. Altered tetrahydrobiopterin metabolism in patients with phenylalanine hydroxylase deficiency.
European journal of pediatrics.
2017 Jul; 176(7):917-924. doi:
10.1007/s00431-017-2932-x. [PMID: 28540433] - Jin-Song Shen, Erland Arning, Michael L West, Taniqua S Day, Shuyuan Chen, Xing-Li Meng, Sabrina Forni, Nathan McNeill, Ozlem Goker-Alpan, Xuan Wang, Paula Ashcraft, David F Moore, Seng H Cheng, Raphael Schiffmann, Teodoro Bottiglieri. Tetrahydrobiopterin deficiency in the pathogenesis of Fabry disease.
Human molecular genetics.
2017 03; 26(6):1182-1192. doi:
10.1093/hmg/ddx032. [PMID: 28158561] - Jianyun Wang, Qianqian Yang, Yaxing Nie, Hao Guo, Fan Zhang, Xueyan Zhou, Xiaoxing Yin. Tetrahydrobiopterin contributes to the proliferation of mesangial cells and accumulation of extracellular matrix in early-stage diabetic nephropathy.
The Journal of pharmacy and pharmacology.
2017 Feb; 69(2):182-190. doi:
10.1111/jphp.12677. [PMID: 28033650] - Jaques Belik, Yulia Shifrin, Erland Arning, Teodoro Bottiglieri, Jingyi Pan, Michelle C Daigneault, Emma Allen-Vercoe. Intestinal microbiota as a tetrahydrobiopterin exogenous source in hph-1 mice.
Scientific reports.
2017 01; 7(?):39854. doi:
10.1038/srep39854. [PMID: 28079055] - Roxana Loperena, David G Harrison. Oxidative Stress and Hypertensive Diseases.
The Medical clinics of North America.
2017 Jan; 101(1):169-193. doi:
10.1016/j.mcna.2016.08.004. [PMID: 27884227] - N Liu, D H Zhao, X L Li, L X Cui, Q H Wu, M Jiang, X D Kong. [PTPS gene analysis and prenatal diagnosis in patients with 6-pyruvoyl-tetra hydropterin synthase deficiency].
Zhonghua fu chan ke za zhi.
2016 Dec; 51(12):890-894. doi:
10.3760/cma.j.issn.0529-567x.2016.12.003. [PMID: 28057123] - Matthew S Alkaitis, Hans C Ackerman. Tetrahydrobiopterin Supplementation Improves Phenylalanine Metabolism in a Murine Model of Severe Malaria.
ACS infectious diseases.
2016 11; 2(11):827-838. doi:
10.1021/acsinfecdis.6b00124. [PMID: 27641435] - Hongchao Wang, Chen Zhang, Jinghan Feng, Yuan Liu, Qin Yang, Haiqin Chen, Zhennan Gu, Hao Zhang, Wei Chen, Yong Q Chen. Role of dihydrofolate reductase in tetrahydrobiopterin biosynthesis and lipid metabolism in the oleaginous fungus Mortierella alpina.
Microbiology (Reading, England).
2016 09; 162(9):1544-1553. doi:
10.1099/mic.0.000345. [PMID: 27488762] - Katja Zschiebsch, Caroline Fischer, Geethanjali Pickert, Annett Häussler, Heinfried Radeke, Sabine Grösch, Nerea Ferreirós, Gerd Geisslinger, Ernst R Werner, Irmgard Tegeder. Tetrahydrobiopterin Attenuates DSS-evoked Colitis in Mice by Rebalancing Redox and Lipid Signalling.
Journal of Crohn's & colitis.
2016 Aug; 10(8):965-78. doi:
10.1093/ecco-jcc/jjw056. [PMID: 26928964] - Ann M Lin, Peizhou Liao, Erin C Millson, Arshed A Quyyumi, Jeanie Park. Tetrahydrobiopterin ameliorates the exaggerated exercise pressor response in patients with chronic kidney disease: a randomized controlled trial.
American journal of physiology. Renal physiology.
2016 05; 310(10):F1016-25. doi:
10.1152/ajprenal.00527.2015. [PMID: 26962106] - Germaine Korner, Tanja Scherer, Dea Adamsen, Alexander Rebuffat, Mark Crabtree, Anahita Rassi, Rossana Scavelli, Daigo Homma, Birgit Ledermann, Daniel Konrad, Hiroshi Ichinose, Christian Wolfrum, Marion Horsch, Birgit Rathkolb, Martin Klingenspor, Johannes Beckers, Eckhard Wolf, Valérie Gailus-Durner, Helmut Fuchs, Martin Hrabě de Angelis, Nenad Blau, Jan Rozman, Beat Thöny. Mildly compromised tetrahydrobiopterin cofactor biosynthesis due to Pts variants leads to unusual body fat distribution and abdominal obesity in mice.
Journal of inherited metabolic disease.
2016 Mar; 39(2):309-19. doi:
10.1007/s10545-015-9909-6. [PMID: 26830550] - Akiko Ohashi, Yusuke Saeki, Tomonori Harada, Masako Naito, Tomihisa Takahashi, Shin Aizawa, Hiroyuki Hasegawa. Tetrahydrobiopterin Supplementation: Elevation of Tissue Biopterin Levels Accompanied by a Relative Increase in Dihydrobiopterin in the Blood and the Role of Probenecid-Sensitive Uptake in Scavenging Dihydrobiopterin in the Liver and Kidney of Rats.
PloS one.
2016; 11(10):e0164305. doi:
10.1371/journal.pone.0164305. [PMID: 27711248] - Shoji Yano, Kathryn Moseley, Xiaowei Fu, Colleen Azen. Evaluation of Tetrahydrobiopterin Therapy with Large Neutral Amino Acid Supplementation in Phenylketonuria: Effects on Potential Peripheral Biomarkers, Melatonin and Dopamine, for Brain Monoamine Neurotransmitters.
PloS one.
2016; 11(8):e0160892. doi:
10.1371/journal.pone.0160892. [PMID: 27513937] - Steven C Wyler, Lauren J Donovan, Mia Yeager, Evan Deneris. Pet-1 Controls Tetrahydrobiopterin Pathway and Slc22a3 Transporter Genes in Serotonin Neurons.
ACS chemical neuroscience.
2015 Jul; 6(7):1198-205. doi:
10.1021/cn500331z. [PMID: 25642596] - Bingjuan Han, Hui Zou, Bingchao Han, Weiwei Zhu, Zhiyang Cao, Yingxia Liu. Diagnosis, treatment and follow-up of patients with tetrahydrobiopterin deficiency in Shandong province, China.
Brain & development.
2015 Jun; 37(6):592-8. doi:
10.1016/j.braindev.2014.09.008. [PMID: 25304915] - Abrar Turki, Gayathri Murthy, Keiko Ueda, Barbara Cheng, Alette Giezen, Sylvia Stockler-Ipsiroglu, Rajavel Elango. Minimally invasive (13)C-breath test to examine phenylalanine metabolism in children with phenylketonuria.
Molecular genetics and metabolism.
2015 Jun; 115(2-3):78-83. doi:
10.1016/j.ymgme.2015.04.005. [PMID: 25943030] - Curtis M Loer, Ana C Calvo, Katrin Watschinger, Gabriele Werner-Felmayer, Delia O'Rourke, Dave Stroud, Amy Tong, Jennifer R Gotenstein, Andrew D Chisholm, Jonathan Hodgkin, Ernst R Werner, Aurora Martinez. Cuticle integrity and biogenic amine synthesis in Caenorhabditis elegans require the cofactor tetrahydrobiopterin (BH4).
Genetics.
2015 May; 200(1):237-53. doi:
10.1534/genetics.114.174110. [PMID: 25808955] - Kathryn D Moseley, Martha J Ottina, Colleen G Azen, Shoji Yano. Pilot study to evaluate the effects of tetrahydrobiopterin on adult individuals with phenylketonuria with measurable maladaptive behaviors.
CNS spectrums.
2015 Apr; 20(2):157-63. doi:
10.1017/s1092852914000455. [PMID: 25323746] - Hendrik Gremmels, Lonneke M Bevers, Joost O Fledderus, Branko Braam, Anton Jan van Zonneveld, Marianne C Verhaar, Jaap A Joles. Oleic acid increases mitochondrial reactive oxygen species production and decreases endothelial nitric oxide synthase activity in cultured endothelial cells.
European journal of pharmacology.
2015 Mar; 751(?):67-72. doi:
10.1016/j.ejphar.2015.01.005. [PMID: 25595727] - Tsin W Yeo, Daniel A Lampah, Enny Kenangalem, Emiliana Tjitra, Ric N Price, J Brice Weinberg, Keith Hyland, Donald L Granger, Nicholas M Anstey. Impaired systemic tetrahydrobiopterin bioavailability and increased dihydrobiopterin in adult falciparum malaria: association with disease severity, impaired microvascular function and increased endothelial activation.
PLoS pathogens.
2015 Mar; 11(3):e1004667. doi:
10.1371/journal.ppat.1004667. [PMID: 25764397] - Matthew P Rubach, Jackson Mukemba, Salvatore Florence, Bert K Lopansri, Keith Hyland, Alicia D Volkheimer, Tsin W Yeo, Nicholas M Anstey, J Brice Weinberg, Esther D Mwaikambo, Donald L Granger. Impaired systemic tetrahydrobiopterin bioavailability and increased oxidized biopterins in pediatric falciparum malaria: association with disease severity.
PLoS pathogens.
2015 Mar; 11(3):e1004655. doi:
10.1371/journal.ppat.1004655. [PMID: 25764173] - Katrin Watschinger, Markus A Keller, Eileen McNeill, Mohammad T Alam, Steven Lai, Sabrina Sailer, Veronika Rauch, Jyoti Patel, Albin Hermetter, Georg Golderer, Stephan Geley, Gabriele Werner-Felmayer, Robert S Plumb, Giuseppe Astarita, Markus Ralser, Keith M Channon, Ernst R Werner. Tetrahydrobiopterin and alkylglycerol monooxygenase substantially alter the murine macrophage lipidome.
Proceedings of the National Academy of Sciences of the United States of America.
2015 Feb; 112(8):2431-6. doi:
10.1073/pnas.1414887112. [PMID: 25675482] - Jeanie Park, Peizhou Liao, Salman Sher, Robert H Lyles, Don D Deveaux, Arshed A Quyyumi. Tetrahydrobiopterin lowers muscle sympathetic nerve activity and improves augmentation index in patients with chronic kidney disease.
American journal of physiology. Regulatory, integrative and comparative physiology.
2015 Feb; 308(3):R208-18. doi:
10.1152/ajpregu.00409.2014. [PMID: 25477424] - Yulan Qi, Diane R Mould, Huiyu Zhou, Markus Merilainen, Donald G Musson. A prospective population pharmacokinetic analysis of sapropterin dihydrochloride in infants and young children with phenylketonuria.
Clinical pharmacokinetics.
2015 Feb; 54(2):195-207. doi:
10.1007/s40262-014-0196-4. [PMID: 25338975] - Pandu Gangula, Kalpana Ravella, Sasanka Chukkapalli, Mercedes Rivera, Shanthi Srinivasan, Ashley Hale, Keith Channon, Janet Southerland, Lakshmyya Kesavalu. Polybacterial Periodontal Pathogens Alter Vascular and Gut BH4/nNOS/NRF2-Phase II Enzyme Expression.
PloS one.
2015; 10(6):e0129885. doi:
10.1371/journal.pone.0129885. [PMID: 26111153] - Andrea Leiva, Bárbara Fuenzalida, Francisco Westermeier, Fernando Toledo, Carlos Salomón, Jaime Gutiérrez, Carlos Sanhueza, Fabián Pardo, Luis Sobrevia. Role for Tetrahydrobiopterin in the Fetoplacental Endothelial Dysfunction in Maternal Supraphysiological Hypercholesterolemia.
Oxidative medicine and cellular longevity.
2015; 2015(?):5346327. doi:
10.1155/2016/5346327. [PMID: 26697136] - Haruo Shintaku, Toshihiro Ohura. Sapropterin is safe and effective in patients less than 4-years-old with BH4-responsive phenylalanine hydrolase deficiency.
The Journal of pediatrics.
2014 Dec; 165(6):1241-4. doi:
10.1016/j.jpeds.2014.08.003. [PMID: 25223838] - Kin Lung Siu, Hua Cai. Circulating tetrahydrobiopterin as a novel biomarker for abdominal aortic aneurysm.
American journal of physiology. Heart and circulatory physiology.
2014 Dec; 307(11):H1559-64. doi:
10.1152/ajpheart.00444.2014. [PMID: 25260610] - Anna Starr, Claire A Sand, Lamia Heikal, Peter D Kelly, Domenico Spina, Mark Crabtree, Keith M Channon, James M Leiper, Manasi Nandi. Overexpression of GTP cyclohydrolase 1 feedback regulatory protein is protective in a murine model of septic shock.
Shock (Augusta, Ga.).
2014 Nov; 42(5):432-9. doi:
10.1097/shk.0000000000000235. [PMID: 25046538] - Sze Ma, Christopher Cheng-Hwa Ma. Recent developments in the effects of nitric oxide-donating statins on cardiovascular disease through regulation of tetrahydrobiopterin and nitric oxide.
Vascular pharmacology.
2014 Nov; 63(2):63-70. doi:
10.1016/j.vph.2014.08.001. [PMID: 25139660] - Junko Takeda, Rieko Nakata, Hiroshi Ueno, Akio Murakami, Mineo Iseki, Masakatsu Watanabe. Possible involvement of a tetrahydrobiopterin in photoreception for UV-B-induced anthocyanin synthesis in carrot.
Photochemistry and photobiology.
2014 Sep; 90(5):1043-9. doi:
10.1111/php.12302. [PMID: 24943195] - M J Reimann, J Häggström, A Mortensen, J Lykkesfeldt, J E Møller, T Falk, L H Olsen. Biopterin status in dogs with myxomatous mitral valve disease is associated with disease severity and cardiovascular risk factors.
Journal of veterinary internal medicine.
2014 Sep; 28(5):1520-6. doi:
10.1111/jvim.12425. [PMID: 25274442] - Andréa E M Stinghen, Jean-Marc Chillon, Ziad A Massy, Agnès Boullier. Differential effects of indoxyl sulfate and inorganic phosphate in a murine cerebral endothelial cell line (bEnd.3).
Toxins.
2014 Jun; 6(6):1742-60. doi:
10.3390/toxins6061742. [PMID: 24902077] - Ana María Gámez-Méndez, Hilda Vargas-Robles, Mónica Arellano-Mendoza, Erika Cruz-Laguna, Amelia Rios, Bruno Escalante. Early stage of obesity potentiates nitric oxide reduction during the development of renal failure.
Journal of nephrology.
2014 Jun; 27(3):281-7. doi:
10.1007/s40620-013-0029-9. [PMID: 24446346] - Livius V d'Uscio, Tongrong He, Anantha Vijay R Santhanam, Li-Jung Tai, Ronald M Evans, Zvonimir S Katusic. Mechanisms of vascular dysfunction in mice with endothelium-specific deletion of the PPAR-δ gene.
American journal of physiology. Heart and circulatory physiology.
2014 Apr; 306(7):H1001-10. doi:
10.1152/ajpheart.00761.2013. [PMID: 24486511] - Hiroki Sato, Mitsugu Uematsu, Wakaba Endo, Tojo Nakayama, Tomoko Kobayashi, Naomi Hino-Fukuyo, Osamu Sakamoto, Haruo Shintaku, Shigeo Kure. Early replacement therapy in a first Japanese case with autosomal recessive guanosine triphosphate cyclohydrolase I deficiency with a novel point mutation.
Brain & development.
2014 Mar; 36(3):268-71. doi:
10.1016/j.braindev.2013.04.003. [PMID: 23660475] - Chunling Wang, Shuyu Zhang, Ying Tian, Baojian Wang, Shuanghe Shen. Effects of simulated heat waves on ApoE-/- mice.
International journal of environmental research and public health.
2014 Jan; 11(2):1549-56. doi:
10.3390/ijerph110201549. [PMID: 24477215] - Teresa D Douglas, Usha Ramakrishnan, Julie A Kable, Rani H Singh. Longitudinal quality of life analysis in a phenylketonuria cohort provided sapropterin dihydrochloride.
Health and quality of life outcomes.
2013 Dec; 11(?):218. doi:
10.1186/1477-7525-11-218. [PMID: 24373161] - Anna E Stanhewicz, Lacy M Alexander, W Larry Kenney. Oral sapropterin acutely augments reflex vasodilation in aged human skin through nitric oxide-dependent mechanisms.
Journal of applied physiology (Bethesda, Md. : 1985).
2013 Oct; 115(7):972-8. doi:
10.1152/japplphysiol.00481.2013. [PMID: 23743404] - Alan Mortensen, Stine Hasselholt, Pernille Tveden-Nyborg, Jens Lykkesfeldt. Guinea pig ascorbate status predicts tetrahydrobiopterin plasma concentration and oxidation ratio in vivo.
Nutrition research (New York, N.Y.).
2013 Oct; 33(10):859-67. doi:
10.1016/j.nutres.2013.07.006. [PMID: 24074744] - Patricia Almudéver, Javier Milara, Alfredo De Diego, Ana Serrano-Mollar, Antoni Xaubet, Francisco Perez-Vizcaino, Angel Cogolludo, Julio Cortijo. Role of tetrahydrobiopterin in pulmonary vascular remodelling associated with pulmonary fibrosis.
Thorax.
2013 Oct; 68(10):938-48. doi:
10.1136/thoraxjnl-2013-203408. [PMID: 23739137] - Anna E Stanhewicz, Lacy M Alexander, W Larry Kenney. Oral sapropterin augments reflex vasoconstriction in aged human skin through noradrenergic mechanisms.
Journal of applied physiology (Bethesda, Md. : 1985).
2013 Oct; 115(7):1025-31. doi:
10.1152/japplphysiol.00626.2013. [PMID: 23869061] - Teresa D Douglas, Hyder A Jinnah, Douglas Bernhard, Rani H Singh. The effects of sapropterin on urinary monoamine metabolites in phenylketonuria.
Molecular genetics and metabolism.
2013 Jul; 109(3):243-50. doi:
10.1016/j.ymgme.2013.04.017. [PMID: 23712020] - Haruo Shintaku, Misao Ohwada. Long-term follow-up of tetrahydrobiopterin therapy in patients with tetrahydrobiopterin deficiency in Japan.
Brain & development.
2013 May; 35(5):406-10. doi:
10.1016/j.braindev.2012.06.010. [PMID: 22832064] - Hongchao Wang, Haiqin Chen, Guangfei Hao, Bo Yang, Yun Feng, Yu Wang, Lu Feng, Jianxin Zhao, Yuanda Song, Hao Zhang, Yong Q Chen, Lei Wang, Wei Chen. Role of the phenylalanine-hydroxylating system in aromatic substance degradation and lipid metabolism in the oleaginous fungus Mortierella alpina.
Applied and environmental microbiology.
2013 May; 79(10):3225-33. doi:
10.1128/aem.00238-13. [PMID: 23503309] - Katrin Watschinger, Ernst R Werner. Alkylglycerol monooxygenase.
IUBMB life.
2013 Apr; 65(4):366-72. doi:
10.1002/iub.1143. [PMID: 23441072] - Jordan D Miller, Yi Chu, Lauren E Castaneda, Kristine M Serrano, Robert M Brooks, Donald D Heistad. Vascular function during prolonged progression and regression of atherosclerosis in mice.
Arteriosclerosis, thrombosis, and vascular biology.
2013 Mar; 33(3):459-65. doi:
10.1161/atvbaha.112.252700. [PMID: 23307875] - Roberto Cerone, Generoso Andria, Marcello Giovannini, Vincenzo Leuzzi, Enrica Riva, Alberto Burlina. Testing for tetrahydrobiopterin responsiveness in patients with hyperphenylalaninemia due to phenylalanine hydroxylase deficiency.
Advances in therapy.
2013 Mar; 30(3):212-28. doi:
10.1007/s12325-013-0011-x. [PMID: 23436109] - Masahito Imanishi, Noriyuki Okada, Yoshio Konishi, Takashi Morikawa, Isseki Maeda, Chizuko Kitabayashi, Masahiro Masada, Nobuo Shirahashi, Christopher S Wilcox, Akira Nishiyama. Angiotensin II receptor blockade reduces salt sensitivity of blood pressure through restoration of renal nitric oxide synthesis in patients with diabetic nephropathy.
Journal of the renin-angiotensin-aldosterone system : JRAAS.
2013 Mar; 14(1):67-73. doi:
10.1177/1470320312454764. [PMID: 22859713] - Geethanjali Pickert, Hee-Young Lim, Andreas Weigert, Annett Häussler, Thekla Myrczek, Maximilian Waldner, Sandra Labocha, Nerea Ferreirós, Gerd Geisslinger, Jörn Lötsch, Christoph Becker, Bernhard Brüne, Irmgard Tegeder. Inhibition of GTP cyclohydrolase attenuates tumor growth by reducing angiogenesis and M2-like polarization of tumor associated macrophages.
International journal of cancer.
2013 Feb; 132(3):591-604. doi:
10.1002/ijc.27706. [PMID: 22753274] - Kristen L Jablonski, Matthew L Racine, Candace J Geolfos, Phillip E Gates, Michel Chonchol, Matthew B McQueen, Douglas R Seals. Dietary sodium restriction reverses vascular endothelial dysfunction in middle-aged/older adults with moderately elevated systolic blood pressure.
Journal of the American College of Cardiology.
2013 Jan; 61(3):335-43. doi:
10.1016/j.jacc.2012.09.010. [PMID: 23141486] - Norbert Weiss, Louisa Papatheodorou, Naoaki Morihara, Robert Hilge, Nagatoshi Ide. Aged garlic extract restores nitric oxide bioavailability in cultured human endothelial cells even under conditions of homocysteine elevation.
Journal of ethnopharmacology.
2013 Jan; 145(1):162-7. doi:
10.1016/j.jep.2012.10.045. [PMID: 23127645] - Peng Kai Ong, Benoît Melchior, Yuri C Martins, Anthony Hofer, Pamela Orjuela-Sánchez, Pedro Cabrales, Graziela M Zanini, John A Frangos, Leonardo J M Carvalho. Nitric oxide synthase dysfunction contributes to impaired cerebroarteriolar reactivity in experimental cerebral malaria.
PLoS pathogens.
2013; 9(6):e1003444. doi:
10.1371/journal.ppat.1003444. [PMID: 23818850] - Philip J Lupo, Claudia Chapa, Darryl Nousome, Cody Duhon, Mark A Canfield, Gary M Shaw, Richard H Finnell, Huiping Zhu. A GCH1 haplotype and risk of neural tube defects in the National Birth Defects Prevention Study.
Molecular genetics and metabolism.
2012 Nov; 107(3):592-5. doi:
10.1016/j.ymgme.2012.09.020. [PMID: 23059057] - Xinrong He, Fuhong Su, Dimitrios Velissaris, Diamantino Ribeiro Salgado, Dalton de Souza Barros, Sophie Lorent, Fabio Silvio Taccone, Jean-Louis Vincent, Daniel De Backer. Administration of tetrahydrobiopterin improves the microcirculation and outcome in an ovine model of septic shock.
Critical care medicine.
2012 Oct; 40(10):2833-40. doi:
10.1097/ccm.0b013e31825b88ba. [PMID: 22846780]