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品質等級
化驗
≥70% (qNMR)
≥95% (TLC)
形狀
powder or crystals
光學活性
[α]/D 15±2°, c = 1 in H2O
雜質
≤20% water
顏色
white to off-white
儲存溫度
2-8°C
SMILES 字串
OC(C[C@@H](C(O)=O)NC(NCCC[C@H](N)C(O)=O)=N)=O.[Li+]
InChI
1S/C10H18N4O6.Li/c11-5(8(17)18)2-1-3-13-10(12)14-6(9(19)20)4-7(15)16;/h5-6H,1-4,11H2,(H,15,16)(H,17,18)(H,19,20)(H3,12,13,14);/q;+1/t5-,6-;/m0./s1
InChI 密鑰
MGJRREWOOITZBB-GEMLJDPKSA-N
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相关类别
生化／生理作用
精氨酸琥珀酸是一种碱性氨基酸，由某些细胞从瓜氨酸、天冬氨酸合成而得，在尿素循环或瓜氨酸-NO循环中可用作精氨酸的前体。该反应是由精氨酸琥珀酸酯合成酶所催化的。精氨酸琥珀酸是柠檬酸循环中通过精氨酸琥珀酸酯裂解酶形成富马酸酯的一种前体。精氨酸琥珀酸裂合酶的缺乏可导致精氨酸琥珀酸裂合酶的缺乏。精氨酸琥珀酸酯（ASA）裂合酶缺乏会导致ASA裂解不良。这会导致细胞中的ASA积聚以及尿液中的ASA过量排泄（精氨琥珀酸尿症）。这种疾病几乎在所有方面都与其他尿素循环缺陷具有相同的特征。ASA裂合酶缺乏的最重要特征是其倾向于在患病个体中引起高氨血症。受影响个体中的ASA肾脏排泄速率几乎等于肾小球滤过率（GFR）。ASA本身是否会因为肝细胞积聚而引起一定程度的毒性尚不可知；这种作用可能有助于解释受影响个体的高氨血症发展。无论如何，该疾病的名称是源于尿液中ASA的快速清除，尽管在血浆中也发现了ASA的水平升高。ASA裂合酶缺乏与高死亡率和高发病率相关。
儲存類別代碼
11 - Combustible Solids
水污染物質分類（WGK）
WGK 3
其他客户在看
Wing Lok Au et al.
Journal of the neurological sciences, 209(1-2), 101-104 (2003-04-11)
A 25-year-old Chinese man presented with a 2-year history of recurrent coma. His plasma ammonia level was extremely elevated, with raised citrulline level and absence of argininosuccinic acid. Adult-onset citrullinaemia, a condition rarely reported outside the Japanese population, was diagnosed.
Pornswan Wasant et al.
Journal of the Medical Association of Thailand = Chotmaihet thangphaet, 85 Suppl 2, S720-S731 (2002-10-31)
Urea Cycle Disorders (UCD) is an inborn error of urea synthesis in which ammonium and other nitrogenous precursors of urea accumulate leading to episodic coma and a high mortality rate. Therapy with peritoneal dialysis, essential amino acids or their nitrogen-free
L D Fleisher et al.
American journal of human genetics, 31(4), 439-445 (1979-07-01)
We have monitored two successive pregnancies in a family which we found to be at risk for argininosuccinic aciduria. We measured argininosuccinic acid (ASA) concentrations in amniotic fluid and utilized an indirect assay of ASA lyase activity in cultured amniotic
V Reid Sutton et al.
Molecular genetics and metabolism, 78(1), 11-16 (2003-02-01)
Argininosuccinate lyase (AL) has several roles in intermediary metabolism. It is an essential component of the urea cycle, providing a pathway for the disposal of excess nitrogen in mammals. AL links the urea cycle to the tricarboxylic acid (TCA) cycle
W J Kleijer et al.
Journal of inherited metabolic disease, 25(5), 399-410 (2002-11-01)
A biochemical variant of argininosuccinate lyase deficiency, found in five individuals, is introduced. In comparison to classical patients, the variant cases of argininosuccinate lyase deficiency were characterized by residual enzyme activity as measured by the incorporation of [14C]citrulline into proteins.
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