所有图片(1)
About This Item
线性分子式:
C6H5CH2CH(NH2)CO2H
CAS号:
分子量:
165.19
Beilstein:
1910408
MDL编号:
UNSPSC代码:
41116107
PubChem化学物质编号:
NACRES:
NA.24
推荐产品
等级
pharmaceutical primary standard
API类
phenylalanine
制造商/商品名称
EDQM
mp
270-275 °C (dec.) (lit.)
应用
pharmaceutical (small molecule)
包装形式
neat
储存温度
2-8°C
SMILES字符串
N[C@@H](Cc1ccccc1)C(O)=O
InChI
1S/C9H11NO2/c10-8(9(11)12)6-7-4-2-1-3-5-7/h1-5,8H,6,10H2,(H,11,12)/t8-/m0/s1
InChI key
COLNVLDHVKWLRT-QMMMGPOBSA-N
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储存分类代码
11 - Combustible Solids
WGK
WGK 1
闪点(°F)
Not applicable
闪点(°C)
Not applicable
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Christineh N Sarkissian et al.
Molecular genetics and metabolism, 86 Suppl 1, S22-S26 (2005-09-17)
Phenylketonuria (PKU) is an autosomal recessive genetic disorder in which mutations in the phenylalanine-4-hydroxylase (PAH) gene result in an inactive enzyme (PAH, EC 1.14.16.1). The effect is an inability to metabolize phenylalanine (Phe), translating into elevated levels of Phe in
Marte I Flydal et al.
IUBMB life, 65(4), 341-349 (2013-03-05)
Mammalian phenylalanine hydroxylase (PAH) catalyzes the rate-limiting step in the phenylalanine catabolism, consuming about 75% of the phenylalanine input from the diet and protein catabolism under physiological conditions. In humans, mutations in the PAH gene lead to phenylketonuria (PKU), and
John J Mitchell et al.
Genetics in medicine : official journal of the American College of Medical Genetics, 13(8), 697-707 (2011-05-11)
Phenylalanine hydroxylase deficiency is an autosomal recessive disorder that results in intolerance to the dietary intake of the essential amino acid phenylalanine. It occurs in approximately 1:15,000 individuals. Deficiency of this enzyme produces a spectrum of disorders including classic phenylketonuria
F J van Spronsen et al.
Journal of inherited metabolic disease, 32(1), 46-51 (2009-02-05)
In phenylketonuria, mental retardation is prevented by a diet that severely restricts natural protein and is supplemented with a phenylalanine-free amino acid mixture. The result is an almost normal outcome, although some neuropsychological disturbances remain. The pathology underlying cognitive dysfunction
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