InChI
1S/C12H21N3O8/c1-4(17)14-8-10(20)9(19)6(3-16)23-11(8)15-7(18)2-5(13)12(21)22/h5-6,8-11,16,19-20H,2-3,13H2,1H3,(H,14,17)(H,15,18)(H,21,22)
SMILES string
CC(=O)NC1C(O)C(O)C(CO)OC1NC(=O)CC(N)C(O)=O
InChI key
YTTRPBWEMMPYSW-UHFFFAOYSA-N
assay
≥98%
form
powder
optical activity
[α]/D 22 to 26 °, c = 1% (w/v) in water
technique(s)
thin layer chromatography (TLC): suitable
storage temp.
−20°C
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Application
n-acetylglucosaminylasparagine [2-Acetamido-1-N-(β-L-aspartyl)-2-deoxy-β-D-glucopyranosylamine] may be useful in studies on Aspartylglycosaminuria.
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
Niki Lindblom et al.
Journal of inherited metabolic disease, 29(5), 637-646 (2006-09-01)
Sleep disturbances are common in many progressive metabolic encephalopathies. The possible presence of disturbed sleep-wake behaviour in the lysosomal storage disorder aspartylglucosaminuria, has not been previously studied, however. The sleep-wake behaviour of 81 patients with aspartylglucosaminuria (AGU, age 3-55 years
M Arvio et al.
Acta neurologica Scandinavica, 112(5), 335-337 (2005-10-13)
To show that the head may shrink in adult patients with aspartylglucosaminuria (AGU), a neurodegenerative disease. The head circumference (HC) of 40 adult patients (age at baseline 15 to 47) was measured twice with an interval of 10 years. Of
U Dunder et al.
FEBS letters, 499(1-2), 77-81 (2001-06-22)
Aspartylglycosaminuria (AGU), a severe lysosomal storage disease, is caused by the deficiency of the lysosomal enzyme, glycosylasparaginase (GA), and accumulation of aspartylglucosamine (GlcNAc-Asn) in tissues. Here we show that human leukocyte glycosylasparaginase can correct the metabolic defect in Epstein-Barr virus
R Guy et al.
Child: care, health and development, 27(2), 173-181 (2001-03-17)
Lysosomal storage diseases are rare and coexistence of more than one in a family can present a diagnostic challenge as illustrated by this study. The index case born to consanguineous Asian parents presented with developmental delay. Investigations led to an
[Aspartylglucosaminuria].
S Yamaguchi
Ryoikibetsu shokogun shirizu, (19 Pt 2)(19 Pt 2), 466-468 (1998-06-30)
商品
N-linked glycosylation, modification, and degradation
N-连接聚糖、修饰和降解
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