H-Abu-OH 是氨基丁酸的三种异构体之一，在患有 Reye 综合征、酪氨酸血症、高胱氨酸尿症、非酮症高甘氨酸血症和鸟氨酸转氨甲酰酶缺乏症的儿童的血浆中升高。
| Cas No. | 1492-24-6 |
| 别名 | L-2-氨基丁酸 |
| 化学名 | (2S)-2-aminobutanoic acid |
| Canonical SMILES | CCC(C(=O)O)N |
| 分子式 | C4H9NO2 |
| 分子量 | 103.1 |
| 溶解度 | Soluble in water or 1% acetic acid |
| 储存条件 | Desiccate at -20℃ |
| General tips | For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while. |
| Shipping Condition | Evaluation sample solution : ship with blue ice
All other available size: ship with RT , or blue ice upon request |
| 产品描述 | H-Abu-OH, one of the three isomers of aminobutyric acid, is elevated in the plasma of children with with Reye's syndrome, tyrosinemia, homocystinuria, nonketotic hyperglycinemia, and ornithine transcarbamylase deficiency. References:
[1]. Yudkoff M, et al. On the clinical significance of the plasma alpha-amino-n-butyric acid:leucine ratio. Am J Clin Nutr. 1979 Feb;32(2):282-5. |
