N-Acetylgalactosamine 6-sulfate
IUPAC 名称
{[(2R,3R,4R,5R)-5-acetamido-2,3,4-trihydroxy-6-oxohexyl]oxy}sulfonic acid
描述
N-Acetylgalactosamine 6-sulfate is found in keratan sulfate and chondroitin 6-sulfate.
PMID: 12489154
Morquio syndrome is caused by an abnormal accumulation of mucopolysaccharides - in this case, keratan sulfate - in the body. Keratan sulfate is excreted in urine.
The symptoms vary from patient to patient, and may include hearing loss, cataracts, skeletal dysplasia, spinal instability, and minor respiratory issues, among others. Two forms are recognized, type A and type B. Type A is a deficiency of the enzyme N-acetylgalactosamine-6-sulfate sulfatase. [HMDB]
异构
CC(=O)N[C@@H](C=O)[C@@H](O)[C@@H](O)[C@H](O)COS(O)(=O)=O
InChI标识符
InChI=1S/C8H15NO9S/c1-4(11)9-5(2-10)7(13)8(14)6(12)3-18-19(15,16)17/h2,5-8,12-14H,3H2,1H3,(H,9,11)(H,15,16,17)/t5-,6+,7+,8-/m0/s1
键
NHZWETQZLORZIR-OSMVPFSASA-N