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A propos de cet article
Formule linéaire :
C6H5CH2CH(NH2)CO2H
Numéro CAS:
Poids moléculaire :
165.19
UNSPSC Code:
41116107
NACRES:
NA.24
PubChem Substance ID:
MDL number:
Beilstein/REAXYS Number:
1910408
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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
SMILES string
N[C@@H](Cc1ccccc1)C(O)=O
InChI key
COLNVLDHVKWLRT-QMMMGPOBSA-N
grade
pharmaceutical primary standard
API family
phenylalanine
manufacturer/tradename
EDQM
mp
270-275 °C (dec.) (lit.)
application(s)
pharmaceutical (small molecule)
format
neat
storage temp.
2-8°C
Catégories apparentées
General description
This product is provided as delivered and specified by the issuing Pharmacopoeia. All information provided in support of this product, including SDS and any product information leaflets have been developed and issued under the Authority of the issuing Pharmacopoeia.For further information and support please go to the website of the issuing Pharmacopoeia.
Application
Phenylalanine EP Reference standard, intended for use in laboratory tests only as specifically prescribed in the European Pharmacopoeia.
Packaging
The product is delivered as supplied by the issuing Pharmacopoeia. For the current unit quantity, please visit the EDQM reference substance catalogue.
Other Notes
Sales restrictions may apply.
Classe de stockage
11 - Combustible Solids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
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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
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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
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
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