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A5707

Sigma-Aldrich

Argininosuccinic acid disodium salt hydrate

≥80%

Synonym(s):

Sodium N-[[[(4S)-4-amino-4-carboxybutyl]amino]iminomethyl]-L-aspartate (2:1)

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About This Item

Empirical Formula (Hill Notation):
C10H16N4Na2O6 · xH2O
CAS Number:
Molecular Weight:
334.24 (anhydrous basis)
MDL number:
UNSPSC Code:
12352100
PubChem Substance ID:
NACRES:
NA.22

Quality Level

Assay

≥80%

form

powder

storage temp.

−20°C

SMILES string

O.[Na+].[Na+].N[C@@H](CCCNC(=N)N[C@@H](CC([O-])=O)C(O)=O)C([O-])=O

InChI

1S/C10H18N4O6.2Na.H2O/c11-5(8(17)18)2-1-3-13-10(12)14-6(9(19)20)4-7(15)16;;;/h5-6H,1-4,11H2,(H,15,16)(H,17,18)(H,19,20)(H3,12,13,14);;;1H2/q;2*+1;/p-2/t5-,6-;;;/m0.../s1

InChI key

UCTAVRCIKYVVFI-VDBFCSKJSA-L

Application

Argininosuccinic acid disodium salt hydrate has been used in the spectrophotometric measurement of argininosuccinate lyase (ASL) enzymatic activity in cell extracts.

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Personal Protective Equipment

dust mask type N95 (US), Eyeshields, Gloves

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Liyan Hu et al.
The Journal of biological chemistry, 288(48), 34599-34611 (2013-10-19)
Argininosuccinic aciduria (ASA) is an autosomal recessive urea cycle disorder caused by deficiency of argininosuccinate lyase (ASL) with a wide clinical spectrum from asymptomatic to severe hyperammonemic neonatal onset life-threatening courses. We investigated the role of ASL transcript variants in

Articles

Inborn errors of metabolism are caused by changes in specific enzymatic reactions and hundreds of different such alterations, which affect about 1 of every 5000 newborns, have been characterized.

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