Document Detail


Characterization of keratan sulfate isolated from liver affected by Morquio syndrome.
MedLine Citation:
PMID:  6222514     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Chemical structures of keratan sulfate (KS) isolated from the liver affected by Morquio syndrome type A (classical type) were investigated. In the KS from Morquio syndrome liver, the molar ratios of hexose, total sulfate, N-sulfate and sialic acid to hexosamine were 5.07, 0.90, 0.18 and 0.08, respectively, and about 10% of hexosamine consisted of galactosamine. The KS resulted in a production of oligosaccharides of relatively larger size after digestion with keratanase, as compared with bovine corneal KS. These findings strongly suggest that KS accumulated in the liver affected by Morquio syndrome may be derived from bony KS.
Authors:
R Minami; T Ikeno; C Igarashi; S Tsugawa; T Nakao
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Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  The Tohoku journal of experimental medicine     Volume:  139     ISSN:  0040-8727     ISO Abbreviation:  Tohoku J. Exp. Med.     Publication Date:  1983 Mar 
Date Detail:
Created Date:  1983-07-08     Completed Date:  1983-07-08     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  0417355     Medline TA:  Tohoku J Exp Med     Country:  JAPAN    
Other Details:
Languages:  eng     Pagination:  321-6     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Animals
Bone and Bones / metabolism
Cattle
Chemical Phenomena
Chemistry
Cornea / analysis
Glycosaminoglycans / isolation & purification*
Humans
Keratan Sulfate / isolation & purification*,  metabolism
Liver / analysis*
Molecular Weight
Mucopolysaccharidosis IV / metabolism*
Chemical
Reg. No./Substance:
0/Glycosaminoglycans; 9056-36-4/Keratan Sulfate

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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