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About This Item
经验公式（希尔记法）:
C12H21NO6 · xLi+
CAS号:
分子量:
275.30 (free acid basis)
分類程式碼代碼:
41116107
PubChem物質ID:
NACRES:
NA.25
推荐产品
product name
谷氨酰-L-肉碱 锂盐, ≥98.0% (TLC)
品質等級
化驗
≥98.0% (TLC)
形狀
solid
光學活性
[α]/D -20±2°, c = 1 in H2O
雜質
≤10% water
顏色
white to off-white
儲存溫度
2-8°C
SMILES 字串
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 密鑰
NXJAXUYOQLTISD-SECBINFHSA-N
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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。
儲存類別代碼
11 - Combustible Solids
水污染物質分類（WGK）
WGK 3
閃點（°F）
Not applicable
閃點（°C）
Not applicable
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Mutations in the glutaryl-CoA dehydrogenase gene can result in Glutaric aciduria type 1(GA 1) by accumulation of glutaric acid, 3-hydroxyglutaric acid (3-OH-GA), and glutarylcarnitine (C5DC). GA 1 is characterized by macrocephaly, subdural hemorrhage (SDH), and dystonic movement disorder after acute
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S I Goodman et al.
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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
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
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
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