Mucopolysaccharidases from Pseudomonas sp.. Isolation and partial characterization of constitutive enzymes involved in the degradation of keratan sulfate and chondroitin sulfate

1986 ◽  
Vol 161 (1) ◽  
pp. 139-147 ◽  
Author(s):  
Denise S. P. Q. HORTON ◽  
Yara M. MICHELACCI
Keyword(s):  
Chondroitin Sulfate ◽  
Keratan Sulfate ◽  
Partial Characterization ◽  
Pseudomonas Sp

Characterization of disease-specific chondroitin sulfate nonreducing end accumulation in mucopolysaccharidosis IVA

Glycobiology ◽  
2020 ◽  
Vol 30 (7) ◽  
pp. 433-445
Author(s):  
Roger Lawrence ◽  
Heather Prill ◽  
Preejith P Vachali ◽  
Evan G Adintori ◽  
Greg de Hart ◽  
...  
Keyword(s):  
Chondroitin Sulfate ◽  
Keratan Sulfate ◽  
Autosomal Recessive Disorder ◽  
Pilot Studies ◽  
Mucopolysaccharidosis Iva ◽  
Enzyme Replacement ◽  
Mps Iva ◽  
Morquio Syndrome ◽  
Disease Specific

Abstract Morquio syndrome type A, also known as MPS IVA, is a rare autosomal recessive disorder caused by deficiency of N-acetylgalactosamine-6-sulfatase, a lysosomal hydrolase critical in the degradation of keratan sulfate (KS) and chondroitin sulfate (CS). The CS that accumulates in MPS IVA patients has a disease-specific nonreducing end (NRE) terminating with N-acetyl-D-galactosamine 6-sulfate, which can be specifically quantified after enzymatic depolymerization of CS polysaccharide chains. The abundance of N-acetyl-D-galactosamine 6-sulfate over other possible NRE structures is diagnostic for MPS IVA. Here, we describe an assay for the liberation and measurement of N-acetyl-D-galactosamine 6-sulfate and explore its application to MPS IVA patient samples in pilot studies examining disease detection, effects of age and treatment with enzyme-replacement therapy. This assay complements the existing urinary KS assay by quantifying CS-derived substrates, which represent a distinct biochemical aspect of MPS IVA. A more complete understanding of the disease could help to more definitively detect disease across age ranges and more completely measure the pharmacodynamic efficacy of therapies. Larger studies will be needed to clarify the potential value of this CS-derived substrate to manage disease in MPS IVA patients.

Partial characterization of the N-linked oligosaccharides occurring on rat hepatocyte glycoproteins

2010 ◽  
Vol 108 (10) ◽  
pp. 323-329 ◽  
Author(s):  
Marti F. A. Bierhuizen ◽  
Moniek de Wit ◽  
Carin A. R. L. Govers ◽  
Willem van Dijk
Keyword(s):  
Partial Characterization ◽  
Rat Hepatocyte

scholarly journals Localization and Partial Characterization of an Aldehydic Component in Tropocollagen

1966 ◽  
Vol 241 (7) ◽  
pp. 1530-1536
Author(s):  
Marcos Rojkind ◽  
Olga O. Blumenfeld ◽  
Paul M. Gallop
Keyword(s):  
Partial Characterization
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