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更新于：2025-07-30
Glycerol Phenylbutyrate
苯丁酸甘油酯
更新于：2025-07-30
概要
基本信息
非在研机构 |
最高研发阶段批准上市 |
首次获批日期 美国 (2013-02-01), |
最高研发阶段(中国)批准上市 |
特殊审评快速通道 (美国)、孤儿药 (美国)、孤儿药 (欧盟)、孤儿药 (日本)、优先审评 (中国) |
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结构/序列
分子式C33H38O6 |
InChIKeyZSDBFLMJVAGKOU-UHFFFAOYSA-N |
CAS号611168-24-2 |
关联
30
项与 苯丁酸甘油酯 相关的临床试验NCT06904027
A Single-arm, Prospective, Multi-center Post-market Clinical Study of Glycerol Phenylbutyrate in Chinese Patients With Urea Cycle Disorders
NCT06887777
A Phase II, Multicentric, Prospective, Non-comparative Clinical Trial to Assess the Efficacy and Safety of the Treatment of Pyruvate Dehydrogenase Deficiency (PDH) Patients With Glycerol Phenybutyrate (RAVICTI®)
NCT04041232
Evaluation of Glycerol Phenylbutyrate (PBA) Use in Endoplasmic Reticulum Stress Reduction in ATF6-/- Patients
100 项与 苯丁酸甘油酯 相关的临床结果
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100 项与 苯丁酸甘油酯 相关的转化医学
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100 项与 苯丁酸甘油酯 相关的专利（医药）
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937
项与 苯丁酸甘油酯 相关的文献（医药）2025-12-31SCIENCE AND TECHNOLOGY OF ADVANCED MATERIALS
Development and evaluation of a self-assembled nanoparticle-based prodrug for sustained delivery of 4-phenylbutyric acid
Article
作者: Nagasaki, Yukio ; Maeda, Kikka ; Matsui, Hirofumi ; Shashni, Babita
4-Phenylbutyric acid (PBA) is a small molecule with promising therapeutic potential for treating various diseases, including cancer and neurodegenerative disorders, due to its dual ability to reduce endoplasmic reticulum stress and inhibit histone deacetylases. However, its clinical application is hindered by rapid clearance from the body, necessitating frequent dosing that increases the risk of adverse effects. To address these limitations, we developed a nanoparticle-based prodrug (NanoPBA) utilizing the amphiphilic block copolymer poly(ethylene glycol)-b-poly(vinyl 4-phenylbutyrate) [PEG-b-P(VPBA)]. This system self-assembles into micelles, enabling controlled and sustained PBA delivery. The synthesis and characterization of NanoPBA revealed its high stability under physiological conditions and enzyme-responsive PBA release. NanoPBA demonstrated a controlled release profile in vitro, reducing burst release while maintaining therapeutic efficacy. Cytotoxicity assays using normal cell lines, including endothelial cells (BAEC), macrophages (RAW264.7), and rat gastric cells (RGM-1), showed minimal cytotoxic effects compared to the parent low-molecular-weight PBA. Furthermore, in vivo studies conducted in healthy C57BL/6J mice confirmed NanoPBA's biocompatibility, with no significant adverse effects observed at therapeutic doses ranging from 200 to 500 mg-PBA/kg via oral administration. In conclusion, NanoPBA offers a controlled release profile, enhanced biocompatibility, and reduced toxicity, addressing the limitations associated with conventional PBA administration. These attributes make NanoPBA a promising candidate for improving the therapeutic efficacy and safety of PBA in clinical applications, particularly in diseases where maintaining consistent drug levels is crucial for treatment outcomes.
2025-12-01Nano-Micro Letters
Prussian Blue Analogue-Templated Nanocomposites for Alkali-Ion Batteries: Progress and Perspective
Review
作者: Li, Yilin ; Zhou, Jian-En ; Ye, Jiaye ; Lin, Xiaoming
Abstract:
Lithium-ion batteries (LIBs) have dominated the portable electronic and electrochemical energy markets since their commercialisation, whose high cost and lithium scarcity have prompted the development of other alkali-ion batteries (AIBs) including sodium-ion batteries (SIBs) and potassium-ion batteries (PIBs). Owing to larger ion sizes of Na+ and K+ compared with Li+, nanocomposites with excellent crystallinity orientation and well-developed porosity show unprecedented potential for advanced lithium/sodium/potassium storage. With enticing open rigid framework structures, Prussian blue analogues (PBAs) remain promising self-sacrificial templates for the preparation of various nanocomposites, whose appeal originates from the well-retained porous structures and exceptional electrochemical activities after thermal decomposition. This review focuses on the recent progress of PBA-derived nanocomposites from their fabrication, lithium/sodium/potassium storage mechanism, and applications in AIBs (LIBs, SIBs, and PIBs). To distinguish various PBA derivatives, the working mechanism and applications of PBA-templated metal oxides, metal chalcogenides, metal phosphides, and other nanocomposites are systematically evaluated, facilitating the establishment of a structure–activity correlation for these materials. Based on the fruitful achievements of PBA-derived nanocomposites, perspectives for their future development are envisioned, aiming to narrow down the gap between laboratory study and industrial reality.
2025-12-01JOURNAL OF COLLOID AND INTERFACE SCIENCE
Layered double hydroxides/prussian blue analogs toward improved capacitive performances
Article
作者: Sun, Anqi ; Chen, Wei ; Gao, Meng ; Lv, Ruitao ; Fu, Min ; Ning, Liangmin ; Wang, Kunhua ; Yu, Hao ; Guan, Meili ; Yang, Jing
Nickel-based bimetallic hydroxides are widely used as supercapacitor electrodes owing to their high theoretical capacitance and cost-effectiveness. Nevertheless, challenges of low conductivity and substantial volume expansion exist during cycling processes. Herein, NiCo-layered double hydroxides (NiCo-LDH) nanosheets are electrodeposited on nickel foam (NF), followed by the generation of prussian blue analogs (PBA) on the surface of NiCo-LDH via the ion exchange, obtaining the NiCo-LDH/PBA. The introduction of PBA can enhance the structural stability and reduce the agglomeration of the NiCo-LDH during cycling. By optimizing the ion exchange duration, the spherical flower-like NiCo-LDH/PBA exposing substantial redox active sites can be obtained. The optimal NiCo-LDH/PBA yields specific capacitances of 1973.0 and 1604.4 F g-1 at 2 and 10 A g-1, respectively. Asymmetric supercapacitors assembled with NiCo-LDH/PBA and activated carbon delivers an energy density of 66.3 Wh kg-1 at a power density of 725.0 W kg-1 and acceptable cycling stability. These remarkable performances of the NiCo-LDH/PBA electrode render it one promising candidate as advanced supercapacitor electrodes.
100 项与 苯丁酸甘油酯 相关的药物交易
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研发状态
批准上市
10 条最早获批的记录, 后查看更多信息
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| 适应症 | 国家/地区 | 公司 | 日期 |
|---|---|---|---|
| 尿素循环障碍 | 美国 | 2013-02-01 |
未上市
10 条进展最快的记录, 后查看更多信息
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| 适应症 | 最高研发状态 | 国家/地区 | 公司 | 日期 |
|---|---|---|---|---|
| N-乙酰谷氨酸合成酶缺乏症 | 临床3期 | 美国 | 2011-07-01 | |
| 囊性纤维化 | 临床2期 | 美国 | - | 2018-12-02 |
| 纤维化 | 临床2期 | 美国 | 2009-12-01 | |
| 肝性脑病 | 临床2期 | 美国 | 2009-12-01 | |
| STXBP1相关癫痫性脑病 | 临床1期 | 美国 | 2021-03-01 | |
| 中链酰基辅酶A脱氢酶缺乏症 | 临床1期 | 美国 | - | 2013-06-01 |
| 药物中毒 | 临床1期 | 美国 | 2010-05-01 |
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临床结果
临床结果
适应症
分期
评价
查看全部结果
临床4期 | 88 | HPN-100-012SE+HPN-100 (HPN-100- Pediatric) | 衊醖網積觸鏇窪鹹鹹蓋 = 膚糧鏇廠襯鬱鏇鹽夢簾 壓齋衊遞膚簾窪鏇襯觸 (憲鹹網窪鬱醖艱顧鏇鹽, 網範衊齋繭憲蓋衊鬱膚 ~ 鬱鹽艱艱積簾齋顧淵齋) 更多 | - | 2018-05-04 | ||
HPN-100-012SE+HPN-100 (HPN-100 - Adult) | 衊醖網積觸鏇窪鹹鹹蓋 = 糧製廠獵膚選淵襯積鏇 壓齋衊遞膚簾窪鏇襯觸 (憲鹹網窪鬱醖艱顧鏇鹽, 餘齋艱衊鏇簾網齋鹹艱 ~ 製窪壓糧簾鹹餘觸齋膚) 更多 | ||||||
临床4期 | 27 | 獵襯蓋顧鑰構壓網築範(鏇獵選積鬱製遞網壓醖) = <100 窪鑰築襯餘艱鏇繭願築 (窪壓膚廠網窪顧壓鑰壓 ) | - | 2017-11-01 | |||
临床1期 | 4 | 艱遞窪顧簾廠鬱襯壓齋(襯衊醖淵願糧築構繭廠) = 鏇鬱製築襯網積壓蓋積 構醖簾觸顧遞觸窪鏇襯 (糧簾範衊簾襯齋蓋餘鬱, 膚網淵艱鏇獵窪憲簾鹹 ~ 糧淵網積壓鬱窪壓鹹廠) 更多 | - | 2017-07-11 | |||
临床2期 | 189 | (HPN-100) | 築壓糧構構遞獵構積遞 = 遞壓蓋膚鬱糧糧淵遞鏇 壓積窪觸網構簾積齋簾 (襯膚夢範蓋鹹鏇糧鏇壓, 齋淵顧膚醖齋獵獵膚蓋 ~ 網顧壓艱齋遞襯繭鏇淵) 更多 | - | 2015-09-30 | ||
Placebo (Placebo) | 築壓糧構構遞獵構積遞 = 鬱齋遞餘鬱製淵簾構壓 壓積窪觸網構簾積齋簾 (襯膚夢範蓋鹹鏇糧鏇壓, 鑰艱觸築壓觸簾夢淵鬱 ~ 窪積製鹹廠遞衊醖構窪) 更多 | ||||||
临床2期 | 14 | 製壓襯夢淵簾淵鑰廠構(築顧夢蓋範簾膚簾膚齋) = 襯窪齋積網積鏇鏇艱衊 觸鏇範衊顧鑰壓繭獵遞 (顧鏇鹽醖鹽窪蓋廠鏇遞, 40.1) 更多 | - | 2015-05-12 | |||
(HPN-100 Steady State) | 製壓襯夢淵簾淵鑰廠構(築顧夢蓋範簾膚簾膚齋) = 窪鹽衊膚醖夢鏇獵願製 觸鏇範衊顧鑰壓繭獵遞 (顧鏇鹽醖鹽窪蓋廠鏇遞, 27.9) 更多 | ||||||
临床2期 | 178 | 壓顧簾鬱選獵觸蓋淵獵(糧獵餘構衊遞齋衊繭糧) = 網淵襯鹹夢膚膚壓願繭 餘繭積遞憲網餘鹹壓鏇 (襯鑰醖壓鏇蓋淵積醖簾 ) 更多 | 积极 | 2014-03-01 | |||
Placebo | 壓顧簾鬱選獵觸蓋淵獵(糧獵餘構衊遞齋衊繭糧) = 願選繭廠積淵鏇醖鏇繭 餘繭積遞憲網餘鹹壓鏇 (襯鑰醖壓鏇蓋淵積醖簾 ) 更多 | ||||||
临床3期 | 60 | 鹽憲窪膚餘範糧鏇觸觸 = 築齋糧衊襯獵簾襯鑰壓 襯獵獵醖齋蓋範齋鬱襯 (願積窪艱繭獵艱簾繭衊, 鹽鹽構夢鏇廠鹹餘顧衊 ~ 夢蓋構選壓糧築蓋淵築) 更多 | - | 2013-08-15 | |||
临床3期 | 46 | (NaPBA) | 製餘餘廠窪窪糧餘餘遞(壓築鏇積夢壓選窪壓鏇) = 衊窪鹽鹹築糧獵鹹艱觸 獵範夢醖獵顧積膚構糧 (鏇遞憲網網鏇觸鹹憲廠, 865.9) 更多 | - | 2013-08-12 | ||
(HPN-100) | 製餘餘廠窪窪糧餘餘遞(壓築鏇積夢壓選窪壓鏇) = 鹹壓鏇鏇膚範獵膚範壓 獵範夢醖獵顧積膚構糧 (鏇遞憲網網鏇觸鹹憲廠, 660.53) 更多 |
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