2-Acetamido-2-deoxy-6-O-alpha-L-fucopyranosyl-D-glucose
IUPAC 名称
N-{3,4,5-trihydroxy-1-oxo-6-[(3,4,5-trihydroxy-6-methyloxan-2-yl)oxy]hexan-2-yl}acetamide
描述
2-Acetamido-2-deoxy-6-O-a-L-fucopyranosyl-D-glucose is an oligosaccharide isolated from the urine of patients with fucosidosis (Fucosidosis is an autosomal recessive lysosomal storage disease caused by defective alpha-L-fucosidase with accumulation of fucose in the tissues. Different phenotypes include clinical features such as neurologic deterioration, growth retardation, visceromegaly, and seizures in a severe early form; coarse facial features, angiokeratoma corporis diffusum, spasticity and delayed psychomotor development in a longer surviving form; and an unusual spondylometaphyseoepiphyseal dysplasia in yet another form. OMIM 230000). (PMID 728478) [HMDB]
异构
CC1OC(OCC(O)C(O)C(O)C(NC(C)=O)C=O)C(O)C(O)C1O
InChI标识符
InChI=1S/C14H25NO10/c1-5-9(19)12(22)13(23)14(25-5)24-4-8(18)11(21)10(20)7(3-16)15-6(2)17/h3,5,7-14,18-23H,4H2,1-2H3,(H,15,17)
键
YBWAUUBLHOFOPK-UHFFFAOYSA-N