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更新于：2026-04-03

Sapropterin Dihydrochloride

盐酸沙丙蝶呤

更新于：2026-04-03

概要

基本信息

药物类型

小分子化药

别名

(6R)-L-erythro-5,6,7,8-tetrahydrobiopterin、(6R)-L-erythro-tetrahydrobiopterin、(−)-(6R)-2-amino-6-((1R,2S)-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4(3H)-pteridinone

+ [33]

靶点

PAH

作用方式

激动剂

作用机制

PAH激动剂(苯丙氨酸羟化酶激动剂)

治疗领域

神经系统疾病遗传病与畸形内分泌与代谢疾病+ [1]

在研适应症

BH4缺乏的B型高苯丙氨酸血症四氢生物蝶呤缺乏症高苯丙氨酸血症+ [2]

非在研适应症

6-丙酮酰四氢蝶呤合酶缺乏症白蛋白尿镰状细胞血症+ [13]

原研机构

Daiichi Sankyo Co., Ltd.

在研机构

APR Applied Pharma Research SA

Dipharma Francis SrlSam OH Pharm Co. Ltd.

+ [5]

非在研机构

Merck KGaA

权益机构

Dr. Reddy's Laboratories Ltd.Asubio Pharma Co. Ltd.

山东新时代药业有限公司

+ [6]

最高研发阶段批准上市

首次获批日期

日本 (1992-03-27),

苯丙酮尿症

最高研发阶段(中国)批准上市

特殊审评孤儿药 (日本)、孤儿药 (韩国)、临床急需境外新药 (中国)、孤儿药 (澳大利亚)

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结构/序列

分子式C9H17Cl2N5O3

InChIKeyRKSUYBCOVNCALL-NTVURLEBSA-N

CAS号69056-38-8

关联

项与盐酸沙丙蝶呤相关的临床试验

NCT07255599

/ Active, not recruiting临床1期IIT

Tetrahydrobiopterin Responsiveness in Phenylketonuria Prediction With the 48-hour Loading Test and Genotype

This study aims to evaluate BH4 responsiveness in our PKU patients and to correlate BH4 responsiveness with their genotype

开始日期2025-09-01

申办/合作机构

Sohag University

NCT06987773

/ Not yet recruiting临床1期

A Pivotal, Phase 1, Randomized, Open-Label, Single-Dose, Two-Way Crossover, Bioequivalence Study of Sapropterin Dihydrochloride 100 mg/mL Oral Suspension (Product Code: RLF-OD032) and Kuvan® (Sapropterin Dihydrochloride) 100 mg Powder for Oral Solution in Healthy Participants Under Fed Conditions

This is a single center, Phase 1, randomized, open-label, single-dose, 2 treatment, 2-period,2-sequence, crossover study designed to compare the pharmacokinetics (PK) of sapropterin between the Test and Reference products in healthy Participants.

开始日期2025-06-30

申办/合作机构

APR Applied Pharma Research SA

NCT06481709

/ Completed临床1期

A Pilot, Phase 1, Randomized, Open-Label, Single-Dose, Four-Way Crossover Study to Compare the Pharmacokinetics of Sapropterin Dihydrochloride 100 mg/mL Oral Suspension (Product Code: RLF-OD032) (Test) With Kuvan® (Sapropterin Dihydrochloride) 100 mg Powder for Oral Solution (Reference) and to Evaluate the Effect of Food and the Effect of Water on the Bioavailability of Sapropterin Dihydrochloride 100 mg/mL Oral Suspension in Healthy Subjects

This is a single center, Phase 1, randomized, open-label, single-dose, 4 treatment, 4-period, 4-sequence, crossover study designed to compare the pharmacokinetics (PK) of sapropterin from the Test and Reference products, and to evaluate the effect of food and the effect of water administration on the bioavailability of sapropterin from the Test product in healthy subjects.
.

开始日期2024-07-30

申办/合作机构

APR Applied Pharma Research SA

100 项与盐酸沙丙蝶呤相关的临床结果

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100 项与盐酸沙丙蝶呤相关的转化医学

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100 项与盐酸沙丙蝶呤相关的专利（医药）

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1,299

项与盐酸沙丙蝶呤相关的文献（医药）

2026-05-01·METABOLISM-CLINICAL AND EXPERIMENTAL

Efficacy and safety of sepiapterin versus sapropterin in patients with phenylketonuria: Results from the Phase 3, randomized, crossover, open-label, active-controlled AMPLIPHY trial

Article

作者: Peters, Heidi ; van Spronsen, Francjan ; Hughes, Catalina ; Bélanger-Quintana, Amaya ; Ingalls, Kim ; Potter, Murray ; Maillot, François ; Smith, Neil ; Tchan, Michel ; Burlina, Alberto ; Inwood, Anita ; Tyčová, Renáta ; Grošelj, Urh ; Vijay, Suresh ; Chakrapani, Anupam ; Rutsch, Frank ; Guilder, Laura ; Venegas-Moreno, Eva Maria ; Giżewska, Maria ; Fjellbirkeland, Olivia ; Zhao, Zhenming ; Muntau, Ania C ; Arnoux, Jean-Baptiste

AIM:

AMPLIPHY is the first Phase 3 study comparing sepiapterin versus sapropterin in children and adults with phenylketonuria (PKU).

METHODS:

AMPLIPHY was an international, Phase 3, two-part, open-label study in participants with PKU aged ≥2 years. Participants responsive to sepiapterin (60 mg/kg/day) in Part 1 (≥20% reduction in blood phenylalanine [Phe]) entered Part 2, a crossover treatment period, and were randomized 1:1 to alternative treatment sequences of sepiapterin (60 mg/kg/day, licensed dosage) and sapropterin (20 mg/kg/day, maximum licensed dosage) for 4 weeks each, with a 14-day washout between treatments. The primary endpoint was mean change in blood Phe from baseline to Weeks 3-4 of each treatment period (Part 2).

RESULTS:

Of 82 participants enrolled, 67 (81.7%) and 62 (75.6%) had reductions in blood Phe ≥20% and ≥30%, respectively, in Part 1. Sixty-two participants were randomized in Part 2 (mean [SD] age, 15.8 [10.8] years). In the primary analysis set (≥30% reduction in blood Phe in Part 1, n = 58), mean (SD) baseline blood Phe before sepiapterin and sapropterin treatment was 725.8 (302.1) and 790.4 (370.0) μmol/L, respectively. Least-squares mean (SE) reduction in blood Phe from baseline was -437.0 (28.0) and -256.6 (28.2) μmol/L, respectively, representing a least-squares mean difference of -180.4 μmol/L (95% CI: -229.5, -131.4; p < 0.0001) and a relative 70% greater reduction with sepiapterin versus sapropterin. Both treatments were well tolerated, with safety profiles consistent with previous reports.

CONCLUSIONS:

Sepiapterin was superior to the highest approved dose of sapropterin in lowering blood Phe. No new safety signals were observed. The trial was registered in the UK Clinical Study Registry, ISRCTN, on January 29, 2024 (ID number, ISRCTN79102999; https://www.isrctn.com/ISRCTN79102999).

2026-03-01·Molecular Therapy-Nucleic Acids

Enhancement of therapeutic transgene insertion for treatment of murine phenylketonuria

Article

作者: Harding, Cary O ; Vonada, Anne ; Baris, Adrian M ; Harper, Laura ; Richards, Daelyn Y ; Winn, Shelley R ; Martinez, Michael A

Low in vivo transgene integration frequency limits the therapeutic efficacy of homology-directed repair (HDR) as a treatment for genetic disorders. This study demonstrates improved efficacy of HDR-mediated gene insertion for the treatment of murine phenylalanine hydroxylase (PAH) deficiency, a model of human phenylketonuria (PKU), through pharmacologic inhibition of competing DNA repair pathways. The targeted integration of a Pah-expressing transgene was enhanced with vanillin, a potent inhibitor of non-homologous end joining (NHEJ), reducing mean serum phenylalanine concentrations by 56.8% in treated mice. This was further improved following co-inhibition of NHEJ and microhomology-mediated end joining (MMEJ), yielding transgene insertions in approximately 10% of genomes with an associated 70.6% decrease in serum phenylalanine. Phenylalanine concentrations were further reduced to 392 μM after oral administration of sapropterin dihydrochloride, a pharmacologic cofactor of PAH. Separately, we demonstrate that rare hepatocytes harboring transgene insertions were successfully expanded to a therapeutically relevant population through selection based upon resistance to acetaminophen toxicity, but this method was hampered by adverse effects upon AAV vector production and the neurologic function of treated neonatal mice related to the presence of shRNA sequences in the AAV vector. These results demonstrate that pharmacologic inhibition of alternative DNA repair pathways can significantly enhance HDR-mediated transgene insertion in vivo.

2026-01-01·MOLECULAR GENETICS AND METABOLISM

Dietetic guidance for nutritional management of people with phenylketonuria receiving sepiapterin

Article

作者: Hollander, Suzanne ; Nagy, Laura ; O'Neill, Sara ; MacDonald, Anita ; Giorda, Sara ; Kogelmann, Christian ; van Wegberg, Annemiek M J ; Pinto, Alex ; Bledsoe, Alexa ; Fujimoto, Hiroki ; Ahring, Kirsten ; Kopesky, Jessica ; Poloni, Soraia ; Roberts, Paige

BACKGROUND/OBJECTIVES:

Phenylketonuria (PKU) is an autosomal recessive inborn error of metabolism. If untreated, elevated blood phenylalanine (Phe) levels lead to neurological and behavioral impairments. Phe levels can be managed through a lifelong Phe-restricted diet; however, this can be challenging to maintain. Sepiapterin is an adjunct therapy for PKU that has shown efficacy in reducing blood Phe levels in both tetrahydrobiopterin (BH₄)-responsive and BH₄-non-responsive people with PKU in clinical trials. Practical guidance is needed for dietitians and other healthcare professionals supporting the dietary management of people with PKU who are initiating or receiving sepiapterin.

METHODS:

A group of international dietitians participated in a questionnaire, virtual meeting, and series of online sessions to develop globally applicable consensus recommendations to support individuals with PKU who are initiating or receiving sepiapterin treatment.

RESULTS:

The expert consensus group has issued 32 recommendations on using sepiapterin in PKU, covering the following topics: preparation, administration, response evaluation, dietary liberalization, protein substitute adjustment, healthy food choices, illness management, and if necessary, treatment cessation. These statements provide a framework to standardize care, clarify therapeutic effectiveness, guide natural protein escalation, reduce low-Phe protein substitutes, and maintain nutritional adequacy. By integrating pharmacological and dietary approaches, the guidance promotes equitable access, supports informed resource allocation, and reinforces the importance of patient-friendly education and ongoing monitoring.

CONCLUSIONS:

This guidance is intended to inform clinical practice and foster consistency in the use of sepiapterin for individuals with PKU. The recommendations should evolve as new scientific evidence and growing clinical experience continue to shape best practice.

188

项与盐酸沙丙蝶呤相关的新闻（医药）

2026-03-26

·德米特医学科技与研究

未来5年，癫痫药物研发最值得关注的10个项目，TDM在列

来源：EP前沿观察癫痫治疗在过去几十年取得了显著进步，但仍有约 30% 的患者属于药物难治性癫痫。因此，全球制药企业和科研机构仍在持续投入大量资源，开发新的抗癫痫治疗策略。未来几年，癫痫药物研发正在出现几个明显趋势：下面介绍未来五年最值得关注的 10个癫痫药物研发项目。第一梯队：新型离子通道调节剂（解决耐药性） 1. Azetukalner (XEN1101) — Xenon Pharmaceuticals 机制： Kv7 钾通道开启剂。看点：它是钾通道赛道的领头羊。目前的 III 期数据显示其对难治性局灶性癫痫具有极强的控制力,且每日只需服药一次,无需滴定。预期：极有可能成为未来 5 年内首个获批的新机制抗癫痫药。 2. BHV-7000 — Biohaven 机制：高选择性 Kv7.2/7.3 调节剂。看点：与 XEN1101 类似,但它在设计上更专注于减少“中枢神经系统副作用”（如嗜睡、头晕）。如果临床数据证实其安全性更高,它将具有极强的市场竞争力。第二梯队：精准医疗与遗传性癫痫（靶向基因） 3. STK-001 — Stoke Therapeutics 机制：反义寡核苷酸（ASO）疗法。看点：针对 Dravet 综合征。它不是单纯控制抽搐,而是通过增加 SCN1A 基因产生的健康蛋白质水平,从底层修复基因缺陷。预期：开启“基因补偿”疗法的新时代。 4. ETX101 — Encoded Therapeutics 机制： AAV 基因疗法。看点：同样针对 Dravet 综合征。这是一种“一劳永逸”的尝试,通过病毒载体将调节序列送入特定神经元。目前正处于早期临床,是全球基因治疗癫痫的风向标。第三梯队：神经炎症与免疫调节（寻找新靶点） 5. Darigabat — Cerevel Therapeutics (已被 AbbVie 收购) 机制：$\alpha 2/3/5$ 选择性 GABA_A 受体正向别构调节剂。看点：传统的安定类药物作用于所有 GABA 受体,导致严重的镇静副作用。Darigabat 避开了引起镇静的 $\alpha 1$ 亚型,试图实现“只抗癫痫、不催眠”的目标。 6. Atuzaginstat — Quince Therapeutics 机制：蛋白酶抑制剂（针对神经炎症）。看点：该药原本研究阿尔茨海默病,但新证据发现其能通过抑制脑部特定细菌代谢物产生的炎症来控制癫痫,是典型的“新靶点”尝试。第四梯队：孤儿药与罕见综合征 7. Soticlestat (TAK-935) — Takeda (武田) 机制：胆固醇 24-羟化酶 (CH24H) 抑制剂。看点：通过调节脑内谷氨酸水平来减少兴奋性。虽然近期在 LGS 的某些终点上表现反复,但在 Dravet 综合征中的潜力依然巨大,是目前唯一的酶抑制剂路径药物。 8. LP352 — Longboard Pharmaceuticals 机制： 5-HT2C 受体超选择性激动剂。看点：针对多种发育性癫痫性脑病（DEE）。它旨在复制芬氟拉明的疗效,同时去除心脏瓣膜损伤的风险。第五梯队：脑机接口与再生医学（终极方案） 9. NRTX-1001 — Neurona Therapeutics 机制：抑制性中间神经元细胞疗法。看点：这是一个革命性的方案——直接向致痫灶移植产生的 GABA 神经元,让它们在那里扎根并抑制异常放电。阶段： I/II 期临床中,部分受试者已实现长期无发作。 10. 智能植入式闭环系统（硬件+软件） — NeuroPace/Medtronic 机制： AI 驱动的响应式神经电刺激（RNS）。看点：虽然不是药物,但其算法更新速度极快。未来 5 年,集成深度学习算法的植入设备将能提前数分钟预测发作并精准放电阻断,这被称为“电子药物”。总结：未来 5 年的三个关键词脱离“钠通道”：研发重心已全面转向钾通道和 GABA 亚型受体。认知挽救：评价一个好药的标准不再仅仅是“发作减少了多少”,而是“孩子的认知好转了多少”。个性化：基因检测将成为癫痫治疗的前置步骤,医生将根据基因报告在上述项目中选择最匹配的武器。这些项目代表了从“改善症状”到“精准对因”甚至“疾病修饰”的范式转变。

2026-03-18

·生物通

2025年12月：肥胖与罕见病治疗领域的新突破、新合作与新疗法

1. 商业新闻 1.1. 收购、合并、授权 1.1.1. BioMarin Pharmaceutical 和 Amicus Therapeutics 罕见病专业公司BioMarin Pharmaceutical宣布收购Amicus Therapeutics，从而扩展其已上市和研发中的酶替代疗法及其他遗传疾病治疗药物组合。此次收购为BioMarin带来了已上市产品migalastat (Galafold?)，一种用于法布里病的口服小分子治疗药物，通过稳定患者体内功能异常的α-半乳糖苷酶起作用；以及用于庞贝病的双组分疗法Pombiliti?/Opfolda?，该疗法可能使对现有酶替代疗法无反应的患者获益。 1.1.2. Mirum Pharmaceuticals 和 Bluejay Therapeutics 另一项罕见病公司收购是Mirum Pharmaceuticals与Bluejay Therapeutics达成的协议。此次收购将扩展Mirum在罕见肝病领域的产品组合，包括鹅去氧胆酸、胆酸和maralixibat。Bluejay的后期研究性产品brelovitug，一种目前处于III期临床试验、用于治疗丁型肝炎感染的全人源单克隆抗体(mAb)，将成为Mirum肝病产品组合的一部分。 1.1.3. Moderna 和 Nanexa 基于其PharmaShell?技术开发长效注射产品的Nanexa公司与mRNA疫苗先驱Moderna签署了技术许可和选择权协议。根据协议，Moderna获得了将其PharmaShell?技术用于其首个选定化合物的许可，并对另外最多四个化合物拥有选择权。两家公司将合作评估PharmaShell?技术调控所选化合物释放速率曲线的潜力。该技术基于原子层沉积，可在药物上沉积厚度高度可控的精确薄金属氧化物薄膜以实现控释。 1.1.4. Shionogi 和 Tanabe Pharma 田边三菱制药发现、开发并商业化了一款用于治疗罕见病肌萎缩侧索硬化症(ALS)的药物依达拉奉。Shionogi现已宣布将收购Radicava?/Radicava ORS?特许经营权，该业务将由Shionogi的一家全资子公司运营，符合其提供罕见病疗法的承诺。 1.1.5. AstraZeneca 和 Jacobio (Jacobs Pharmaceutical) KRAS蛋白作为胰腺癌、结直肠癌和肺癌的靶点日益受到关注。阿斯利康获得了由Jacobio发现的泛KRAS抑制剂JAB-23E73在中国以外所有地区的独家开发和商业化权利。Jacobio已在中国和美国将该化合物推进至I期研究，早期研究表明了抗肿瘤活性迹象。 1.1.6. Ipsen 和 Simcere Zaiming Ipsen通过一项交易获得了Simcere Zaiming发现的靶向LRRC15的抗体偶联药物(ADC) SIM0613的权利，为其产品组合增加了另一款ADC。该ADC的抗体部分靶向LRRC15，该蛋白在肉瘤、胶质母细胞瘤和黑色素瘤等间充质来源的肿瘤中过表达。Ipsen获得了中国以外所有地区的开发、生产和商业化权利。 1.2. 合作 1.2.1. Regeneron 和 Tessera Therapeutics Tessera Therapeutics开发了一种体内基因书写疗法TSRA-196，作为一次性治疗，可以纠正单个基因突变，从而持久恢复患者肝脏中α-1抗胰蛋白酶(AAT)的产生。再生元制药宣布将与Tessera合作推进该疗法，双方将分担开发成本和未来的潜在利润。 1.2.2. Pfizer 和 YaoPharma 辉瑞宣布与上海复星医药的子公司YaoPharma就其小分子GLP-1受体激动剂YP05002达成合作和许可协议，该药物正处于慢性体重管理的I期临床试验中。在YaoPharma完成I期试验后，辉瑞将承担进一步开发工作，并拥有该交易的制造和商业化权利。 1.2.3. Crescent biopharma 和 Kelun-Biotech Crescent Biopharma与科伦博泰宣布建立合作伙伴关系，共同开发和商业化肿瘤治疗药物，包括新型药物组合。具体而言，两家公司旨在推进Crescent的早期PD-1 × VEGF双特异性抗体CR-001和科伦博泰的I期准备就绪的ADC SKB105，后者是一种靶向整合素β-6 (ITGB6)、携带细胞毒性拓扑异构酶I抑制剂有效载荷的抗体。 1.2.4. Zealand pharma 和 OTR therapeutics Zealand Pharma与剂泰医药建立了多项目战略合作，Zealand将利用其在肥胖和代谢健康方面的专业知识，并借助剂泰医药的小分子发现能力，针对代谢疾病的多个靶点发现和开发新型疗法。 1.2.5. Harbor BioMed 和 Bristol Myers Squibb 百时美施贵宝宣布与和铂医药建立全球战略合作，以发现和开发下一代多特异性抗体，作为免疫学和肿瘤学治疗药物。此次合作还可能利用和铂医药在中国快速开展早期临床试验的能力。 1.3. 其他商业新闻 1.3.1. 默克皮下注射派姆单抗在德国上市因知识产权纠纷受阻 Halozyme与Alteogen之间关于其专有人重组透明质酸酶技术的专利纠纷有了新进展。德国法院批准了Halozyme的请求，对默克发出初步禁令，责令其停止分销和提供其免疫肿瘤药物派姆单抗的皮下制剂。法院认定其即将侵犯Halozyme的一项MDASE?技术专利。 2. 产品批准及其他监管新闻 2.1. 新型运动病预防疗法获美国批准 (tradipitant胶囊, Nereus?, Vanda Pharmaceuticals) 四十多年来首个新机制的运动病治疗药物tradipitant (Nereus?)获得了美国食品药品监督管理局(FDA)的批准。Tradipitant是一种神经激肽-1 (NK-1)受体拮抗剂。Vanda计划在2026年初推出该药用于运动病。 2.2. 自用速效治疗异常心律疗法获批 (etripamil鼻喷雾剂, Cardamyst?, Milestone Therapeutics) Milestone Therapeutics宣布其快速起效的钙拮抗剂etripamil (Cardamyst?)鼻喷雾剂获美国FDA批准，用于治疗阵发性室上性心动过速(PSVT)的急性症状发作。该药允许患者在发作时自行给药，快速恢复正常心律。 2.3. 新型第三代PCSK9抑制剂获批帮助患者实现降胆固醇目标 (lerodalcibep皮下注射剂, lerochol?, LIB Therapeutics) LIB Therapeutics的第三代PCSK9抑制剂lerodalcibep (Lerochol?)皮下注射剂获得了美国FDA批准。该药是一种基于adnectin的化合物，作为饮食和运动的辅助手段，用于降低患有高胆固醇血症（包括杂合子型家族性高胆固醇血症(FH)）的成年人的低密度脂蛋白胆固醇(LDL-C)。与目前的PCSK9产品相比，该药可能为患者提供每月注射一次（预计2026年底推出自动注射器版本）、且在家储存时无需冷藏（可在室温下保存长达三个月）的优势。 2.4. 超长效生物制剂治疗呼吸系统疾病在欧盟获推荐批准并在英美获批 (depemokimab皮下注射剂, exdensur?, GSK) 葛兰素史克(GSK)的超长效靶向白细胞介素-5 (IL-5)的单抗depemokimab (Exdensur?)本月获得了多项监管进展。欧洲药品管理局(EMA)人用药品委员会(CHMP)推荐其批准作为12岁及以上、患有2型炎症（以嗜酸性粒细胞水平升高为特征）的严重哮喘患者的附加维持治疗。该药在英国和美国也获得了相应批准。该药非常方便，每年仅需给药两次。 2.5. 离体基因疗法治疗罕见病Wiskott-Aldrich综合征获美国批准 (etuvetigene autotemcel输注液, waskyra?, Fondazione Telethon) 非营利性生物医学组织Fondazione Telethon的离体基因疗法etuvetigene autotemcel输注液(Waskyra?)获得了美国FDA批准，用于治疗Wiskott-Aldrich综合征(WAS)。该疗法包含用携带WAS基因拷贝的慢病毒载体体外转导的自体CD34+细胞和祖细胞。 2.6. SMA基因疗法新配方获批用于老年患者 (onasemnogene abeparvovec鞘内注射剂, itvisma?, Novartis) 诺华公司用于治疗脊髓性肌萎缩症(SMA)的基因替代疗法onasemnogene abeparvovec的新配方获得了美国FDA批准，该配方设计用于治疗2岁及以上、携带确诊SMN1基因突变的儿童、青少年和成人。新配方(itvisma?)设计用于鞘内给药，允许使用更小的剂量和体积，但可靶向中枢神经系统。 2.7. 皮下剂型双特异性抗体获批，与靶向酪氨酸激酶抑制剂联用可实现无化疗一线治疗方案 (amivantamab/透明质酸酶皮下注射剂, rybrevant Fastpro?; Lazertinib, Lazcluze?, Johnson & Johnson) 强生公司的amivantamab与透明质酸酶产品(Rybrevant Faspro?)与lazertinib (Lacluze?)联合，作为一线疗法的一部分，获得了美国FDA批准。此次批准的amivantamab皮下制剂使治疗更加简便，给药时间从静脉给药的数小时缩短至约五分钟。 2.8. 皮下剂型B细胞/T细胞接合双特异性抗体治疗滤泡性淋巴瘤在美国获批 (mosunetuzumab皮下注射剂, lunsumio VELO?, Genentech) 基因泰克公司的mosunetuzumab皮下注射剂(Lunsumio VELO?)获美国FDA批准，作为Lunsumio?静脉注射剂的替代方案，用于治疗既往接受过两种或以上全身治疗、出现复发或难治性的滤泡性淋巴瘤(FL)成人患者。与大多数其他近期批准的静脉转皮下单抗制剂不同，该药不需要与透明质酸酶共配方。 2.9. 首个口服GLP-1受体激动剂治疗肥胖症获批 (semaglutide片剂, Wegovy?, Novo Nordisk) 美国FDA批准了首个口服给药的GLP-1受体激动剂用于治疗肥胖症，即诺和诺德公司的semaglutide (Wegovy?)片剂。该药通过与渗透促进剂salcaprozate sodium (SNAC)共配方，实现口服给药后的全身可利用度。 2.10. 症状性梗阻性肥厚型心肌病新疗法获美国监管批准 (aficamten片剂, Myqorzo?, Cytokinetics) Cytokinetics公司的口服心肌肌球蛋白抑制剂aficamten片剂(Myqorzo?)获得了美国FDA批准，用于治疗症状性梗阻性肥厚型心肌病。该药与现有药物mavacamten同属一类，通过抑制心肌肌球蛋白马达活性来改善心脏功能。 3. 临床试验 3.1. 口服小分子STAT6蛋白降解剂在特应性皮炎中显示出与现有生物制剂相当的阳性结果 (KT-621, Kymera Therapeutics) Kymera Therapeutics公布了其首创、每日一次、口服给药的STAT6蛋白降解剂KT-621在中重度特应性皮炎患者中的Ib期研究结果。所有相关生物标志物的变化以及有利的临床终点均显示出与目前用于该适应症的单抗dupilumab相当的效果。 3.2. 口服小分子GLP-1受体激动剂推进至III期试验 (aleniglipron, Structure Therapeutics) Structure Therapeutics公布了其口服生物可利用的小分子GLP-1受体激动剂aleniglipron的IIb期试验阳性数据。该药提供了剂量依赖性的体重减轻，且耐受性特征与其他GLP-1受体激动剂相似。公司计划在2026年中启动III期研究。 3.3. 临床疗效证明III期试验中眼内双维甲酸积累抑制剂治疗Stargardt病有效 (tinlarebant, Belite Bio) Belite Bio公布了其药物tinlarebant治疗青少年Stargardt病1型(STGD1)的III期研究的阳性顶线结果。该药通过减少进入眼睛的视黄醇量，从而降低有毒的A2E水平，抑制STGD1的进展。研究中，接受治疗的患者视网膜病变显著减少。 3.4. 治疗皮肤结节性基底细胞癌的微针阵列疗法II期试验完成入组 (D-MNA, 多柔比星微针阵列, Medicus Pharma) Medicus Pharma开发了一种基于可溶解聚合物的微针阵列装置，用于非侵入性治疗皮肤基底细胞癌(BCC)。该装置含有阿霉素，可直接杀伤细胞并诱导免疫反应以防止疾病复发。公司宣布其正在进行的II期试验已完成90名受试者的目标入组，顶线结果预计在2026年第一季度公布。 3.5. 早期试验数据显示基因疗法治疗Dravet综合征结果积极 (ETX101, Encoded Therapeutics) Encoded Therapeutics报告了其基因疗法ETX101治疗与SCN1A基因变异相关的Dravet综合征患儿的I/II期研究的积极中期结果。在耐药参与者中，在七个多月的观察期内，第三高剂量组患者的可计数月癫痫发作频率持续、剂量依赖性降低了78%。 3.6. 外用二氢睾酮阻滞剂治疗男性型脱发的III期研究显示显著结果 (clascoterone外用溶液, Cosmo Pharmaceuticals) Cosmo Pharmaceuticals报告了其5% clascoterone外用溶液制剂治疗男性雄激素性脱发（男性型脱发）的III期试验结果。结果显示，与安慰剂相比，目标区域毛发数量显著增加，且治疗安全性可与安慰剂相比。 3.7. 细胞疗法在治疗杜氏肌营养不良症中显示出获益 (CAP-1002, deramiocel, Capricor Therapeutics) Capricor Therapeutics完成了其细胞疗法deramiocel治疗杜氏肌营养不良症的额外III期临床研究。接受活性药物治疗的参与者在上肢功能和心肌病方面，与安慰剂治疗受试者相比，显示出显著的改善（延缓进展）。这些结果将提交给美国FDA。 3.8. 宣布CAR T细胞疗法治疗多发性骨髓瘤的II期试验阳性数据 (anitocabtagene autoleucel, Kite/Arcellx) Kite Oncology宣布了其靶向BCMA的CAR T细胞疗法anitocabtagene autoleucel在复发或难治性多发性骨髓瘤患者中的关键II期研究结果。在这个重度经治人群中，74%的治疗受试者达到了完全缓解，且无延迟性神经毒性。 3.9. 僵硬人综合征细胞疗法II期试验受试者活动能力改善且对助行器依赖减少 (KYV-101, mivocabtagene autoleucel, Kyverna Therapeutics) Kyverna Therapeutics宣布了其CAR T细胞疗法mivocabtagene autoleucel (KYV-101)治疗罕见、进行性自身免疫性神经系统疾病僵硬人综合征(SPS)的注册性II期试验结果。单次剂量的治疗为25英尺步行时间等指标带来了稳健且持续的改善，并减少了对抗标签免疫疗法或挽救性疗法的需求。 3.10. 从注射肽类GLP-1受体激动剂转为口服小分子激动剂后，III期试验受试者体重减轻得以维持 (orforglipron, Lilly) 礼来公司报告了一项III期临床研究的结果，该研究中，先前接受皮下注射semaglutide或tirzepatide治疗72周以实现体重减轻的受试者，转而接受口服orforglipron或安慰剂治疗。52周后，在两种注射疗法后改用orforglipron治疗，在体重减轻维持方面优于安慰剂。 3.11. 首创三重激素受体激动剂在晚期试验中带来显著体重减轻和骨关节炎疼痛缓解 (retatrutide, Lilly) 礼来公司公布了其下一代减重疗法retatrutide的III期临床试验消息。Retatrutide是一种可注射的生物疗法，作为单一分子，可激动与代谢疾病相关的三种受体：GLP-1受体、胃抑制多肽(GIP)受体和胰高血糖素受体。在患有肥胖和膝骨关节炎但无糖尿病的成人中进行的试验显示，在最高剂量下体重显著减轻了28.7%，同时骨关节炎疼痛减轻了74.3%。 3.12. 下一代TYK2抑制剂治疗斑块状银屑病的关键临床试验结果前景看好 (TAK-279, zasocitinib, Takeda) 武田公司公布了两项III期研究的结果，评估其下一代高选择性口服酪氨酸激酶2 (TYK2)抑制剂zasocitinib用于中重度斑块状银屑病成人患者的疗效。在武田报告的研究中，到治疗第16周，超过一半的活性药物治疗受试者达到了皮肤完全清除或几乎完全清除。

2026-03-12

·Business Wire

Otsuka Unveils New Repinatrabit Open-Label Extension Data in Phenylketonuria (PKU) Signaling Efficacy in Adolescents at the 2026 American College of Medical Genetics and Genomics (ACMG) Meeting

PRINCETON, N.J. & TOKYO--(BUSINESS WIRE)--Otsuka Pharmaceutical Development & Commercialization, Inc. and Otsuka Pharmaceutical Co., Ltd. (Otsuka) today announced new open-label extension study data for repinatrabit (JNT-517), demonstrating early and clinically meaningful reductions in blood phenylalanine (Phe) levels (-67% mean change from baseline) in adolescents with phenylketonuria (PKU). These new data, along with the study design for the pivotal global Phase 3 PheORD trial (NCT06971731) in adults, were presented at the 2026 American College of Medical Genetics and Genomics (ACMG) meeting. “These data mark an important milestone in our efforts to advance the understanding and treatment of PKU,” said John Kraus, M.D., Ph.D., executive vice president and chief medical officer, Otsuka. “Maintaining control of blood Phe levels poses a critical challenge for adolescent patients, with higher levels leading to clinical symptoms such as executive function impairment and other intellectual disabilities. Repinatrabit represents a novel investigational approach with the potential to reduce Phe levels and broaden treatment options for the PKU community.” In a long-term, open-label extension, the first cohort of adolescents receiving repinatrabit 75 mg twice daily – the lower of the two doses evaluated in the Phase 2 adolescent study (NCT06637514) – achieved sustained Phe reductions at the first timepoint that could be evaluated in the open‑label setting (Day 56 / OLE Month 1). At this assessment, adolescents achieved an average –67% reduction from baseline. The parent Phase 2 trial that patients are rolling over from remains blinded. Early time points in the long-term open-label extension are embargoed to protect the integrity (i.e., blinding) of the parent study. Additional blinded early‑treatment data from the randomized period will be presented in the future. The scale and consistency of Phe reduction observed in adolescents were comparable to those reported in adult Phase 2 studies, reinforcing the strong potential of repinatrabit across diverse age groups1. All participants in the cohort demonstrated a clinical response, including adolescents with prior sapropterin experience (both responders and non-responders), as well as one sapropterin-naïve participant. Repinatrabit was well tolerated, with a safety profile consistent with prior adult Phase 2 findings. No new treatment-related adverse events were identified, and no serious adverse events were reported. The study of this novel therapeutic strategy for pediatric PKU is ongoing, with patients in the open-label extension advancing to the higher dose (150 mg) of repinatrabit in the second cohort. Otsuka is also advancing the pivotal Phase 3 PheORD trial in adults with PKU2. The global, randomized, double-blind, placebo-controlled study is designed to evaluate the efficacy and safety of two oral dose regimens of repinatrabit (75 mg or 150 mg twice daily) compared with placebo2. In the first treatment period, approximately 120 patients will be randomized in a 1:1:1 ratio to one of the two repinatrabit dose groups or placebo for six weeks, with the primary endpoint assessing short-term mean reduction in blood Phe levels at Weeks 2, 4 and 62. During the secondary study period, participants originally assigned to repinatrabit will continue on their respective dose for an additional 46 weeks, while those originally assigned to placebo will transition to repinatrabit 150 mg twice daily, with continued safety and efficacy follow-up through 52 weeks2. The trial intentionally enrolls a broad adult PKU population across disease severity and prior treatment experience, reflecting real-world clinical practice2. Completion of the primary endpoint of the adult population in the Phase 3 study is anticipated in late 2026, with full study completion expected in 2028. About Repinatrabit Repinatrabit (JNT-517) is an investigational, selective, small-molecule inhibitor of the Phe transporter SLC6A19 that has the potential to be a first-in-class oral therapy used to treat any person with PKU, regardless of age or genotype3. Repinatrabit acts at a novel, cryptic allosteric site to block kidney reabsorption of Phe and offers a novel investigational approach to reduce blood Phe levels3. Repinatrabit was developed by Jnana Therapeutics, which became a wholly owned subsidiary of Otsuka in September 2024. It was created using Jnana’s proprietary and innovative drug discovery approach, the RAPID platform. Repinatrabit has received orphan drug designation and rare pediatric disease designation from the U.S. Food and Drug Administration (FDA) for the treatment of PKU. Otsuka is actively recruiting in the global Phase 3 PheORD clinical trial (NCT06971731) to assess the efficacy, safety and tolerability of oral Repinatrabit in participants with PKU2. About Phenylketonuria Phenylketonuria (PKU) is a disorder caused by pathogenic variants in the phenylalanine hydroxylase (PAH) gene, rendering the enzyme unable to metabolize Phe and leading to its toxic accumulation in the body, including the brain4. PKU affects approximately 1 in 24,000 individuals globally, including approximately 1 in 10,000 individuals in the United States4. If left untreated, PKU can adversely affect brain development and lead to intellectual disability and other serious health problems4. High levels of Phe have been associated with neurological and psychiatric presentations such as executive function impairment, headaches, sleep disorders, anxiety, and depression5,6. Maintaining control of blood Phe levels remains one of the most significant challenges for people living with PKU, particularly during adolescence, a critical period of neurodevelopment when elevated Phe levels can contribute to cognitive and psychiatric complications7. About Otsuka Otsuka Pharmaceutical Co., Ltd. is a total healthcare company that focuses on each individual's potential to enhance their well-being. Our medical-related business provides treatments and diagnostics for both physical and mental health. Our nutraceutical business supports daily health maintenance and improvement. Otsuka's unique products and services are based on scientific evidence, under the guidance of our corporate philosophy: Otsuka-people creating new products for better health worldwide. Otsuka America Pharmaceutical, Inc. and Otsuka Pharmaceutical Development & Commercialization, Inc. are the US-based indirect subsidiaries of the global healthcare company Otsuka Pharmaceutical Co. Ltd. Otsuka’s US companies share a deep commitment to the development and commercialization of innovative products in the spaces of neuroscience, nephrology, and immunology. At our core is perseverance—a fierce determination to overcome any obstacle, regardless of setbacks, on behalf of patients, caregivers, and their loved ones. We will not be bound by doing what’s been done before. Learn more at www.otsuka-us.com. References Fernanda Leal-Pardinas, Nicola Longo, Cary O. Harding, Ania C. Muntau, Rani H. Singh, Andreu Viader, George Vratsanos, Haoling H. Weng, Elaina Jurecki, Wei Li, Guissou Dabiri, John P. Throup, Markey McNutt. Phase 2 Efficacy of Repinatrabit in Reducing Plasma Phenylalanine (Phe) Concentration in Adults with Phenylketonuria (PKU): Subgroup Analyses by Baseline Characteristics. 2026 NPKUA Scientific Conference. O. Harding C., et al. Design of a Randomized, Double-Blind, Placebo-Controlled, Phase 3, Efficacy and Safety Trial of Repinatrabit in Adults with Phenylketonuria. Acmg.planion.com. Accessed March 5, 2026. https://acmg.planion.com/Web.User/AbstractDet?ACCOUNT=ACMG&ABSID=876450&CONF=AM26&ssoOverride=OFF&CKEY=6543N084O . Cary Harding, Nicola Longo, Andreu Viader, Toby Vaughn, Fernanda Leal-Pardinas, Elaina Jurecki, Markey McNutt, Ania Muntau, Rani Singh, Joel Barrish, George Vratsanos, Haoling Weng, John Throup, O01: Efficacy and safety outcomes of JNT-517, a first-in-class SLC6A19 inhibitor, in adults with phenylketonuria: A randomized study, Genetics in Medicine Open,Volume 3, Supplement 2, 2025, 101964, ISSN 2949-7744, https://doi.org/10.1016/j.gimo.2025.101964 . Hillert A, Anikster Y, Belanger-Quintana A, Burlina A, Burton BK, Carducci C, Chiesa AE, Christodoulou J, Đorđević M, et al. The Genetic Landscape and Epidemiology of Phenylketonuria. Am J Hum Genet. 2020 Aug 6;107(2):234-250. doi:10.1016/j.ajhg.2020.06.006. Epub 2020 Jul 14. PMID: 32668217; PMCID: PMC7413859. Ashe K;Kelso W;Farrand S;Panetta J;Fazio T;De Jong G;Walterfang M; Psychiatric and cognitive aspects of phenylketonuria: The limitations of diet and promise of new treatments. Frontiers in psychiatry. Accessed March 5, 2026. https://pubmed.ncbi.nlm.nih.gov/31551819/ . Gassi R., et al. Prevalence of sleep disorders in early-treated phenylketonuric children and adolescents. Correlation with dopamine and serotonin status. ScienceDirect. Accessed March 5, 2026. https://www.sciencedirect.com/science/article/pii/S109037981930176X . G; R. Phenylketonuria as an adherence disease. Patient preference and adherence. 2025. Accessed March 5, 2026. https://pubmed.ncbi.nlm.nih.gov/40248132/ .

临床结果临床2期临床3期孤儿药

100 项与盐酸沙丙蝶呤相关的药物交易

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外链

KEGG	Wiki	ATC	Drug Bank
D01798	盐酸沙丙蝶呤	A16AX07	DB00360

研发状态

批准上市

10 条最早获批的记录,

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适应症	国家/地区	公司	日期
BH4缺乏的B型高苯丙氨酸血症	欧盟	Dipharma Francis Srl	2022-02-16
BH4缺乏的B型高苯丙氨酸血症	冰岛	Dipharma Francis Srl	2022-02-16
BH4缺乏的B型高苯丙氨酸血症	列支敦士登	Dipharma Francis Srl	2022-02-16
BH4缺乏的B型高苯丙氨酸血症	挪威	Dipharma Francis Srl	2022-02-16
四氢生物蝶呤缺乏症	澳大利亚	BioMarin Pharmaceutical, Inc.	2010-10-28
高苯丙氨酸血症	欧盟	BioMarin International Ltd.	2008-12-02
高苯丙氨酸血症	冰岛	BioMarin International Ltd.	2008-12-02
高苯丙氨酸血症	列支敦士登	BioMarin International Ltd.	2008-12-02
高苯丙氨酸血症	挪威	BioMarin International Ltd.	2008-12-02
苯丙酮尿症	日本	Daiichi Sankyo Co., Ltd.	1992-03-27

未上市

10 条进展最快的记录,

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适应症	最高研发状态	国家/地区	公司	日期
神经精神综合征	临床3期	美国	BioMarin Pharmaceutical, Inc.	2010-08-01
神经精神综合征	临床3期	加拿大	BioMarin Pharmaceutical, Inc.	2010-08-01
自闭症	临床3期	美国	BioMarin Pharmaceutical, Inc.	2009-08-01
Lesch-Nyhan综合征	临床3期	美国	BioMarin Pharmaceutical, Inc.	2009-04-01
母源性苯丙酮尿症	临床3期	美国	BioMarin Pharmaceutical, Inc.	2006-02-01
白蛋白尿	临床2期	美国	BioMarin Pharmaceutical, Inc.	2008-05-01
慢性肾病	临床2期	美国	BioMarin Pharmaceutical, Inc.	2008-05-01
内皮功能障碍	临床2期	美国	BioMarin Pharmaceutical, Inc.	2007-09-01
镰状细胞血症	临床2期	美国	BioMarin Pharmaceutical, Inc.	2007-05-01
间歇性跛行	临床2期	美国	BioMarin Pharmaceutical, Inc.	2006-12-01

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临床结果

适应症

分期

评价

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研究	分期	人群特征	评价人数	分组	结果	评价	发布日期


NCT06481709 (CTgov)	临床1期	-	16	sapropterin dihydrochloride (Treatment A)	製齋鬱積夢艱網獵構簾(醖淵遞繭廠夢齋積鹽廠) = 壓範範艱積鬱壓顧憲壓鹽鏇淵顧窪壓繭夢願艱 (製鏇醖膚鹽選製範餘艱, 548.82) 更多	-	2025-06-12
				sapropterin dihydrochloride (Treatment B)	製齋鬱積夢艱網獵構簾(醖淵遞繭廠夢齋積鹽廠) = 淵糧齋淵鬱衊遞鬱壓遞鹽鏇淵顧窪壓繭夢願艱 (製鏇醖膚鹽選製範餘艱, 258.17) 更多
NCT01114737 (Pubmed \| J Pediatr)	临床3期	苯丙酮尿症	86	Sapropterin	鏇憲餘鬱齋齋網糧願壓(蓋廠窪齋餘蓋築膚壓網) = 積網淵鹽膚製顧廠繭積鹹積願壓鑰築鬱衊願蓋 (網淵壓遞糧範遞壓廠鑰 ) 更多	积极	2023-05-30
				Placebo	獵鏇蓋製夢夢廠鬱簾鬱(鑰艱築獵膚蓋膚衊壓廠) = 積繭鹹鬱鑰鏇憲積艱選廠選醖簾鏇夢淵壓醖窪 (餘壓艱繭鏇鏇簾繭膚簾 )
NCT02677870 (CTgov)	临床4期	苯丙酮尿症	7	Diet treatment (Diet Treatment)	餘淵顧艱廠壓膚製網鹹 = 壓鑰糧繭壓簾製顧壓齋壓窪鏇齋壓糧選襯遞糧 (願壓構夢網蓋鬱艱齋憲, 簾鹽選齋構膚選窪壓齋 ~ 繭艱膚觸鹹窪簾醖艱襯) 更多	-	2022-04-27
				Diet treatment+saproterin dihydrochloride (Standard Dose Saproterin Dihydrochloride)	餘淵顧艱廠壓膚製網鹹 = 鹹獵齋齋鏇壓膚鑰醖衊壓窪鏇齋壓糧選襯遞糧 (願壓構夢網蓋鬱艱齋憲, 製壓壓蓋顧鑰蓋選顧醖 ~ 獵鹹醖願襯範顧襯鑰積) 更多
NCT01376908 (SPARK, Pubmed \| Orphanet J Rare Dis) 人工标引	临床3期	苯丙酮尿症	51	sapropterin (extension' group)	鹽襯鬱鏇蓋蓋築淵齋遞(醖選繭餘簾鹽壓顧鬱齋) = 膚遞製糧憲憲膚鑰鬱築鹽窪夢簾簾膚餘範製膚 (衊積憲醖鹽獵衊顧鹽鑰, 2.8 ~ 13.8)	积极	2021-08-03
				sapropterin (continuous' group)	鹽襯鬱鏇蓋蓋築淵齋遞(醖選繭餘簾鹽壓顧鬱齋) = 憲蓋鹽遞窪積鏇願願餘鹽窪夢簾簾膚餘範製膚 (衊積憲醖鹽獵衊顧鹽鑰, 28.9 ~ 48.6)
NCT00532844 (CTgov)	临床2期	内皮功能障碍	52	Sapropterin Dihydrochloride (Sapropterin Dihydrochloride)	窪鹹膚蓋憲繭淵鹽夢鑰(壓夢衊獵積範選積網獵) = 憲廠鹽築構夢衊鏇願淵願顧積網築醖鬱鏇範襯 (鏇餘齋鬱範襯築簾鑰膚, 455) 更多	-	2021-05-17
				Vitamin C+Sapropterin Dihydrochloride (Sapropterin Dihydrochloride+Vitamin C)	窪鹹膚蓋憲繭淵鹽夢鑰(壓夢衊獵積範選積網獵) = 繭餘襯廠憲蓋繭觸遞網願顧積網築醖鬱鏇範襯 (鏇餘齋鬱範襯築簾鑰膚, 376) 更多
NCT00445978 (CTgov)	临床2期	镰状细胞血症	32	Sapropterin Dihydrochloride	淵選糧淵鑰鬱衊簾範鹽(窪憲願觸遞夢選壓襯鹹) = 餘遞繭觸夢繭憲壓糧獵簾簾積醖鹽顧衊築壓衊 (廠廠膚窪糧遞範構鏇選, 0.4292) 更多	-	2021-01-28
NCT00403494 (CTgov)	临床2期	间歇性跛行 \| 外周动脉疾病	190	Sapropterin Dihydrochloride (Sapropterin Dihydrochloride)	壓醖糧醖齋淵製鬱積淵(繭選範鹹鬱願膚鏇獵壓) = 鑰鬱艱選願膚繭選廠積鏇窪範膚鬱窪鹽窪襯鏇 (壓顧鹹觸鬱壓鑰製醖鏇, 2.803) 更多	-	2021-01-07
				Placebo (Placebo)	壓醖糧醖齋淵製鬱積淵(繭選範鹹鬱願膚鏇獵壓) = 願範廠鏇製襯繭艱鹽獵鏇窪範膚鬱窪鹽窪襯鏇 (壓顧鹹觸鬱壓鑰製醖鏇, 2.876) 更多
NCT01439555 (CTgov)	临床2期	阿尔茨海默症	11	simvastatin+Tetrahydrobiopterin+L-Arginine	齋觸獵鏇窪鬱鏇齋築鑰(壓醖鹽顧遞鏇築齋壓鏇) = 築憲繭製蓋範構觸淵鬱鏇繭窪壓築襯壓窪選夢 (鹹夢簾積鏇積淵製鬱壓, 0.116513988) 更多	-	2019-07-30
NCT01706965 (CTgov)	临床1/2期	分裂情感性障碍 \| 精神分裂症	27	Kuvan (Kuvan)	鑰鬱壓齋築艱簾構築糧(襯網憲遞願醖窪簾簾壓) = 繭衊選餘願範遞遞簾餘鹹範鹽鑰簾鬱獵鏇醖獵 (鹽襯鏇壓網範鑰襯衊簾, 10.9) 更多	-	2017-09-13
				Multivitamin (Multivitamin)	鑰鬱壓齋築艱簾構築糧(襯網憲遞願醖窪簾簾壓) = 艱願簾簾繭鑰膚壓築壓鹹範鹽鑰簾鬱獵鏇醖獵 (鹽襯鏇壓網範鑰襯衊簾, 13.7) 更多
NCT00802893 (CTgov)	临床2期	内皮功能障碍 \| 高血压	3	placebo+6R-BH4	繭簾築積壓膚襯遞選醖(選糧膚壓餘選獵淵積窪) = 觸製範製顧遞積齋醖獵鑰顧選廠膚憲蓋鹹蓋壓 (壓衊廠鏇齋鏇鏇蓋糧製, 製蓋鏇艱鑰鏇衊醖淵齋 ~ 繭製製繭網製顧壓築構) 更多	-	2017-08-21