所有图片(1)
About This Item
经验公式（希尔记法）:
C12H21NO6 · xLi+
CAS号:
分子量:
275.30 (free acid basis)
UNSPSC代码:
41116107
PubChem化学物质编号:
NACRES:
NA.25
推荐产品
产品名称
谷氨酰-L-肉碱 锂盐, ≥98.0% (TLC)
质量水平
方案
≥98.0% (TLC)
表单
solid
旋光性
[α]/D -20±2°, c = 1 in H2O
杂质
≤10% water
颜色
white to off-white
储存温度
2-8°C
SMILES字符串
C[N+](C)(C)C[C@H](OC(CCCC(O)=O)=O)CC([O-])=O.C
InChI
1S/C12H21NO6/c1-13(2,3)8-9(7-11(16)17)19-12(18)6-4-5-10(14)15/h9H,4-8H2,1-3H3,(H-,14,15,16,17)/t9-/m1/s1
InChI key
NXJAXUYOQLTISD-SECBINFHSA-N
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相关类别
生化/生理作用
戊二酰肉碱的形成和排泄升高是戊二酰辅酶A脱氢酶缺乏症所导致的结果，而这是一种赖氨酸和色氨酸代谢的先天性错误。由于戊二酰肉碱的形成和尿排泄升高所引起的继发肉碱消耗可能在戊二酰辅酶A脱氢酶缺乏症的神经病变中起重要作用，从而诱发兴奋性神经毒性和线粒体功能障碍。
储存分类代码
11 - Combustible Solids
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
Stefan Kölker et al.
Annals of neurology, 55(1), 7-12 (2004-01-06)
Glutaryl-CoA dehydrogenase deficiency is an inherited organic aciduria with predominantly neurological presentation. Biochemically, it is characterized by an accumulation and enhanced urinary excretion of two key organic acids, glutaric acid and 3-hydroxyglutaric acid. If untreated, acute striatal degeneration is often
K Kidouchi et al.
Clinica chimica acta; international journal of clinical chemistry, 164(3), 261-266 (1987-05-15)
A technique for the identification of glutarylcarnitine in urine from a patient with glutaric aciduria type 1 is described. The patient's urine sample was partially purified using an anion exchange column and analyzed by a carboxylic acid analyzer fitted with
Kim M Huffman et al.
Diabetes care, 32(9), 1678-1683 (2009-06-09)
To determine whether circulating metabolic intermediates are related to insulin resistance and beta-cell dysfunction in individuals at risk for type 2 diabetes. In 73 sedentary, overweight to obese, dyslipidemic individuals, insulin action was derived from a frequently sampled intravenous glucose
Glutaric aciduria; a "new" disorder of amino acid metabolism.
S I Goodman et al.
Biochemical medicine, 12(1), 12-21 (1975-01-01)
Chee-Seng Lee et al.
Metabolic brain disease, 28(1), 61-67 (2012-10-30)
Glutaric aciduria type I (GA-I) is an inborn error of lysine and tryptophan metabolism. Clinical manifestations of GA-I include dystonic or dyskinetic cerebral palsy, but when the symptoms occur, treatment is not effective. In Taiwan, newborn screening for GA-I started
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