72715
谷氨酰-L-肉碱 锂盐
≥98.0% (TLC)
别名:
L-肉碱谷氨酸锂
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关于此项目
经验公式（希尔记法）:
C12H21NO6 · xLi+
化学文摘社编号:
分子量:
275.30 (free acid basis)
UNSPSC Code:
41116107
PubChem Substance ID:
NACRES:
NA.25
产品名称
谷氨酰-L-肉碱 锂盐, ≥98.0% (TLC)
SMILES string
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
assay
≥98.0% (TLC)
form
solid
optical activity
[α]/D -20±2°, c = 1 in H2O
impurities
≤10% water
color
white to off-white
storage temp.
2-8°C
Quality Level
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Biochem/physiol Actions
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。
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
Hee Su Kim et al.
Annals of clinical and laboratory science, 44(2), 213-216 (2014-05-06)
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
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
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
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