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- 详细信息
- 文献和实验
- 技术资料
- 库存：
有现货
- 英文名：
2-Ketobutyric acid
- CAS号：
600-18-0
- 保质期：
根据瓶身LOT查询
- 供应商：
浙江羽翔生物科技有限公司
- 保存条件：
2-8°C
- 规格：
5G
属性：
质量水平：100
检测方案：97%
形式：liquid
Bp：84 °C/20 mmHg (lit.)
Mp：30-34 °C (lit.)
储存温度：2-8°C
SMILES字符串：CCC(=O)C(O)=O
应用：
2-酮丁酸可作为反应物合成：
通过酶催化胺化反应生成
(S)-2-氨基丁酸。
在乙醇环境下通过活细胞反应生成
L-异亮氨酸。
在硫酸环境下通过 1,2-二氨 ji 苯生成 3-乙基-2-喹 lin 醇。
在生物催化剂环境下使用 2,3-二氨基萘生成
3-乙苯[g]喹喔啉-2(1H)-酮。
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文献和实验Signatures of cell stress and altered bioenergetics in skin fibroblasts from patients with multiple sclerosis.
Multiple sclerosis (MS) is a central nervous system inflammatory demyelinating disease and the most common cause of non-traumatic disability in young adults. Despite progress in the treatment of the active relapsing disease, therapeutic options targeting irreversible progressive decline remain limited. Studies using skin fibroblasts derived from patients with neurodegenerative disorders demonstrate that cell stress pathways and bioenergetics are altered when compared to healthy individuals. However, findings in MS skin fibroblasts are limited. Here, we collected skin fibroblasts from 24 healthy control individuals, 30 patients with MS, and ten with amyotrophic lateral sclerosis (ALS) to investigate altered cell stress profiles. We observed endoplasmic reticulum swelling in MS skin fibroblasts, and increased gene expression of cell stress markers including BIP, ATF4, CHOP, GRP94, P53, and P21. When challenged against hydrogen peroxide, MS skin fibroblasts had reduced resiliency compared to ALS and controls. Mitochondrial and glycolytic functions were perturbed in MS skin fibroblasts while exhibiting a significant increase in lactate production over ALS and controls. Our results suggest that MS skin fibroblasts have an underlying stress phenotype, which may be disease specific. Interrogating MS skin fibroblasts may provide patient specific molecular insights and aid in prognosis, diagnosis, and therapeutic testing enhancing individualized medicine.
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