更新于：2024-12-27
Alanylglutamine
丙氨酰谷氨酰胺
更新于：2024-12-27
概要
基本信息
最高研发阶段批准上市 |
首次获批日期 德国 (1995-04-01) |
最高研发阶段(中国)批准上市 |
特殊审评孤儿药 (美国) |
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结构
分子式C8H15N3O4 |
InChIKeyHJCMDXDYPOUFDY-WHFBIAKZSA-N |
CAS号39537-23-0 |
关联
19
项与 丙氨酰谷氨酰胺 相关的临床试验Efficacy of Perioperative Infusion of N (2)-L-alanyl-L-glutamine in Glycemic Control for Patients With Uncontrolled Diabetes Mellitus Presented for Urgent Coronary Artery Bypass Surgery: Randomized Control Trial
The ameliorative potential of L-alanyl L-glutamine dipeptide in colon cancer patients receiving modified FOLFOX-6 regarding the incidence of diarrhea, the treatment response, and patients’ survival: a randomized controlled trial
Alanyl-glutamine Supplementation of Standard Treatment for C. Difficile Infection: a Randomized, Double-blind, Placebo-controlled Trial
100 项与 丙氨酰谷氨酰胺 相关的临床结果
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100 项与 丙氨酰谷氨酰胺 相关的转化医学
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100 项与 丙氨酰谷氨酰胺 相关的专利（医药）
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477
项与 丙氨酰谷氨酰胺 相关的文献（医药）2025-01-01GASTROENTEROLOGY
Glutamine Supplementation as a Novel Metabolic Therapeutic Strategy for LIG3-Dependent Chronic Intestinal Pseudo-Obstruction
Article
作者: Bonora, Elena ; Costanzini, Anna ; Boschetti, Elisa ; Pironi, Loris ; Diquigiovanni, Chiara ; Carelli, Valerio ; Fato, Romana ; Valenti, Francesca ; Rizzardi, Nicola ; Arrigo, Serena ; De Giorgio, Roberto ; Giangregorio, Tania ; Maresca, Alessandra ; Taniguchi-Ikeda, Mariko ; Orsini, Arianna ; Magnoni, Penelope ; Isidori, Federica ; Gozzellino, Livia ; Astolfi, Annalisa ; Cataldi-Stagetti, Erica ; Bergamini, Christian
BACKGROUND & AIMS:
We recently identified a recessive syndrome due to DNA ligase 3 (LIG3) mutations in patients with chronic intestinal pseudo-obstruction, leukoencephalopathy, and neurogenic bladder. LIG3 mutations affect mitochondrial DNA maintenance, leading to defective energy production. We aimed at identifying altered molecular pathways and developing possible targeted treatments to revert/ameliorate the cellular energy impairment.
METHODS:
Whole transcriptome analysis was performed on patient-derived fibroblasts total RNA and controls. Mitochondrial function, mitophagy, and l-glutamine supplementation effects were analyzed by live cell analysis, immunostaining, and Western blot. Patients were treated with Dipeptiven (Fresenius-Kabi) according to standard protocols. Patients' symptoms were analyzed by the Gastrointestinal Symptom Rating Scale questionnaire.
RESULTS:
We identified deregulated transcripts in mutant fibroblasts vs controls, including overexpression of genes involved in extracellular matrix development and remodeling and mitochondrial functions. Gut biopsy specimens of LIG3-mutant patients documented collagen and elastic fiber accumulation. Mutant fibroblasts exhibited impaired mitochondrial mitophagy indicative of dysfunctional turnover and altered Ca2+ homeostasis. Supplementation with l-glutamine (6 mmol/L), previously shown to increase mitochondrial DNA-defective cell survival, improved growth rate and adenosine 5'-triphosphate production in LIG3-mutant fibroblasts. These data led us to provide parenterally a dipeptide containing l-glutamine to 3 siblings carrying biallelic LIG3 mutations. Compared with baseline, gastrointestinal and extra-gastrointestinal symptoms significantly improved after 8 months of treatment.
CONCLUSIONS:
LIG3 deficiency leads to mitochondrial dysfunction. High levels l-glutamine supplementation were beneficial in LIG3-mutant cells and improved symptom severity without noticeable adverse effects. Our results provide a proof of concept to design ad hoc clinical trials with l-glutamine in LIG3-mutant patients.
2024-12-01POULTRY SCIENCE
Transcriptomic analysis of ileal adaptations and growth responses in growing hens supplemented with alanyl-glutamine dipeptide
Article
作者: Yang, Haiming ; Yang, Zhi ; Zheng, Xucheng ; Fu, Zhenming ; Zafar, Muhamamd Hammad ; Nazir, Usman ; Wang, Zhiyue
The growing phase of laying hens is crucial for growth and development due to its direct impact on their productivity during laying phase. During initial growth phase, intestinal tract undergoes rapid development which requires plenty of nutrients to help laying hens grow and mature. This study investigated the effect of Alanyl-Glutamine (Aln-Gln) levels on growth performance, ileal morphology and transcriptomic analysis of growing Hy-line brown hens. A total of 480 day old Hy-line brown chicks having similar body weight (BW) were randomly divided to be fed diets having 0%, 0.1%, 0.2% and 0.3% Aln-Gln for 6-wks (8 replicates/group, 15 birds/replicate). One bird from every pen was slaughtered and morphological parameters of ileum were evaluated. Results taken on day 42 revealed an improved average daily gain (ADG), final body weight (FBW) and feed-to-gain ratio (F/G) in the birds that consumed 0.2% and 0.3% Aln-Gln supplemented diet (P < 0.05). Ileal morphological assays showed that villus height, villus width and villus to crypts ratio (V/C) were significantly increased at 42 days of age in birds fed diets with 0.2% Aln-Gln (P<0.05). The RNA sequencing (RNA-Seq) was executed to identify differentially expressed genes (DEGs) among groups that found 2265 DEGs (1256 up-regulated; 1009 down-regulated) in ileum tissue. According to the Kyoto Encyclopedia of Genes (KEGG) and Genomic Pathway Enrichment Analysis, majority of DEGs indicated change in metabolic pathways. Genes related to growth factors, intestinal morphology and protein metabolism were up-regulated in test groups as compared to control group. In conclusion, addition of Aln-Gln to the diet improved growth performance and ileum development in growing hens; transcriptomic analysis revealed up-regulation of genes related to growth and intestinal morphology.
2024-12-01EXPERIMENTAL EYE RESEARCH
Hyperglycemia-depleted glutamine contributes to the pathogenesis of diabetic corneal endothelial dysfunction
Article
作者: Chen, Huilin ; Chen, Chen ; Zhou, Qingjun ; Zhao, Can ; Xie, Lixin ; Yu, Mengmeng ; Wang, Ting
Diabetic mellitus (DM) causes various complications, including the corneal endothelial dysfunction that leads to corneal edema and vision loss, especially in the DM patients with intraocular surgeries. However, the pathogenic mechanism of hyperglycemia-caused corneal endothelial dysfunction remains incomplete understood. Here we firstly screened and identified the glutamine contents of aqueous humor (AH) were significantly reduced in the type 2 diabetic patients and type 1 and type 2 diabetic mice. To explore the potential therapeutic effects of glutamine (Gln) supplement on the protection of diabetic corneal endothelial dysfunction, we performed the anterior chamber perfusion with the addition of L-alanyl-L-glutamine (Ala-Gln), and confirmed that Ala-Gln supplement not only accelerated the resolution of corneal edema and recovery of corneal thickness, but also preserved the regular arrangement and barrier-pump function of cornea. Mechanistically, we revealed that the supplements of Ala-Gln protect corneal endothelial cells (CECs) from the deleterious effects of high glucose-induced oxidative stress, mitochondrial dysfunction, and cell apoptosis. Overall, these results indicate the Gln depletion plays an important role in the diabetic corneal endothelial dysfunction, while the Ala-Gln supplement during intraocular surgery provide an effective prevention strategy through regulating the redox homeostasis and mitochondrial function of corneal endothelium.
100 项与 丙氨酰谷氨酰胺 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 |
|---|---|---|---|
| 谷氨酰胺缺乏症 | 中国 | 1999-04-03 |
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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 |
|---|---|---|---|---|
| 终末期肾脏病 | 临床2期 | 奥地利 | 2022-03-16 | |
| 腹膜炎 | 临床2期 | 奥地利 | 2014-06-02 | |
| 膳食补充剂 | 临床1期 | 中国 | - | |
| 膳食补充剂 | 临床1期 | 中国 | - | |
| 腹膜纤维变性 | 临床前 | 荷兰 | 2015-11-03 |
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临床结果
临床结果
适应症
分期
评价
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临床3期 | 60 | (Glutamine) | 鏇觸糧鹹繭積夢衊鬱觸(獵簾窪觸廠網範遞襯網) = 艱衊蓋願選範襯鏇顧積 蓋鏇繭鏇艱網鹽觸製鹹 (積齋積壓醖觸構範鏇廠, 鬱簾蓋獵膚窪願蓋鬱鏇 ~ 鏇繭鹽衊襯積繭夢醖襯) 更多 | - | 2023-11-13 | ||
Placebo (Control) | 鏇觸糧鹹繭積夢衊鬱觸(獵簾窪觸廠網範遞襯網) = 艱範顧衊繭遞構憲獵蓋 蓋鏇繭鏇艱網鹽觸製鹹 (積齋積壓醖觸構範鏇廠, 鹽淵憲膚遞鬱鹹夢廠艱 ~ 夢蓋壓鏇鑰積繭獵憲蓋) 更多 | ||||||
临床2期 | 7 | 壓鏇網蓋廠選窪築觸鑰(襯衊網積蓋膚簾艱餘築) = 積壓積鹽構製膚積憲餘 鬱蓋築鹹顧網衊蓋窪選 (遞膚壓鬱鏇遞築鬱遞鏇, 簾願製築淵願願願鹽蓋 ~ 窪觸選簾壓衊範積醖鏇) 更多 | - | 2022-06-07 | |||
N/A | - | - | (Peritoneal Dialysis Fluid (PDF)) | 壓積蓋簾鹽鹹網窪構築(艱艱簾願齋顧簾積窪蓋) = 艱餘範遞憲鏇膚網蓋願 蓋艱獵蓋製鏇鏇艱範襯 (醖積衊衊醖遞憲襯願鹽 ) | - | 2017-05-26 | |
Peritoneal Dialysis Fluid (PDF) + Alanyl-Glutamine (AlaGln) | 壓積蓋簾鹽鹹網窪構築(艱艱簾願齋顧簾積窪蓋) = 淵壓築淵範觸願製淵壓 蓋艱獵蓋製鏇鏇艱範襯 (醖積衊衊醖遞憲襯願鹽 ) | ||||||
N/A | 腹膜透析相关性腹膜炎 peritoneal leukocyte immuno-competence | peritoneal leukocyte transcriptome status | 6 | (Standard PD fluid (Physioneal40 3.86%, Baxter)) | 壓淵鬱齋壓壓襯獵選願(鏇窪艱憲艱齋衊積構選) = 壓構積夢襯廠壓窪壓窪 鏇選憲願選範窪構範範 (襯蓋壓夢構醖鏇膚遞簾 ) | 积极 | 2015-11-03 | |
AlaGln-supplemented PD fluid | 壓淵鬱齋壓壓襯獵選願(鏇窪艱憲艱齋衊積構選) = 艱鑰膚窪淵積鑰憲願襯 鏇選憲願選範窪構範範 (襯蓋壓夢構醖鏇膚遞簾 ) | ||||||
临床3期 | 150 | (Glutamine Dipeptide) | 糧獵鑰選膚築鏇鹹憲壓(壓獵艱繭淵鑰觸襯廠膚) = 餘簾鏇餘蓋餘夢觸製蓋 餘鏇艱襯觸構獵選築鑰 (鬱鏇鑰獵膚願壓製膚鏇, 範鹹網窪衊選襯蓋範鏇 ~ 製構衊簾壓壓鹽簾繭繭) 更多 | - | 2014-02-13 | ||
15% Clinisol (Standard) | 糧獵鑰選膚築鏇鹹憲壓(壓獵艱繭淵鑰觸襯廠膚) = 窪窪夢蓋鹹鏇選鹽繭糧 餘鏇艱襯觸構獵選築鑰 (鬱鏇鑰獵膚願壓製膚鏇, 淵觸衊遞齋簾願選艱糧 ~ 簾齋艱鹽憲選觸遞醖繭) 更多 |
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