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The urine of five patients with three distinct diseases ("I Cell disease" and two new types of mucolipidosis) contains sialic acid-rich oligosaccharides in a high amount: 50 to 500 fold the normal. The structure of the major components are as follows alpha-NANA-(2 leads to 6)-beta-Gal-(1 leads to 4)-beta-GlcNAc-(1 leads to 2)-alpha-Man-(1 leads to 3)-beta-Man-(1 leads to 4)-GlcNAc, [alpha-NANA-(2 leads to 6) beta-Gal-(1 leads to 4)-beta-Glc-NAc-(1 leads to 2)-alpha-Man-(1 leads to 3) [beta-Gal-(1 leads to 4)-beta-GlcNAc-(1 leads to 2)-alpha-Man-(1 leads to 6)]-beta-Man-(1 leads to 4)-GlcNAc and alpha-NANA-(2 leads to 6)-beta-Gal-(1 leads to 4)-beta-GlcNAc-(1 leads to 2)-alpha-Man-(1 leads to 3)[alpha-NANA-(2 leads to 6)-beta-Gal-(1 leads to 4)-beta-GlcNAc-(1 leads to 2)-alpha-Man-(1 leads to 6)[-beta-Man-(1 leads to 4)-GlcNAC. These results suggest that a dieficit in alpha-neuraminidase is associated to these three different disorders and that an endo-beta-D-N-acetyglucosaminidase is able to release sialyloligosaccharides by splitting the sialyglycans of glycoproteins.
G Stecker, and
B Fournet, and
T HondiAssah, and
G Spik, and
J Montreuil, and
P Maroteaux, and
P Durand, and
J P Farriaux
May 1977,
European journal of biochemistry,
G Stecker, and
B Fournet, and
T HondiAssah, and
G Spik, and
J Montreuil, and
P Maroteaux, and
P Durand, and
J P Farriaux
September 1976,
Biochimica et biophysica acta,
G Stecker, and
B Fournet, and
T HondiAssah, and
G Spik, and
J Montreuil, and
P Maroteaux, and
P Durand, and
J P Farriaux
February 1978,
European journal of biochemistry,
G Stecker, and
B Fournet, and
T HondiAssah, and
G Spik, and
J Montreuil, and
P Maroteaux, and
P Durand, and
J P Farriaux
May 1983,
European journal of biochemistry,
G Stecker, and
B Fournet, and
T HondiAssah, and
G Spik, and
J Montreuil, and
P Maroteaux, and
P Durand, and
J P Farriaux
October 1985,
Journal of biochemistry,
G Stecker, and
B Fournet, and
T HondiAssah, and
G Spik, and
J Montreuil, and
P Maroteaux, and
P Durand, and
J P Farriaux
February 2012,
Anales de pediatria (Barcelona, Spain : 2003),
G Stecker, and
B Fournet, and
T HondiAssah, and
G Spik, and
J Montreuil, and
P Maroteaux, and
P Durand, and
J P Farriaux
February 1975,
Orvosi hetilap,
G Stecker, and
B Fournet, and
T HondiAssah, and
G Spik, and
J Montreuil, and
P Maroteaux, and
P Durand, and
J P Farriaux
March 1973,
Journal de radiologie, d'electrologie, et de medecine nucleaire,
G Stecker, and
B Fournet, and
T HondiAssah, and
G Spik, and
J Montreuil, and
P Maroteaux, and
P Durand, and
J P Farriaux
January 1973,
Archives francaises de pediatrie,
G Stecker, and
B Fournet, and
T HondiAssah, and
G Spik, and
J Montreuil, and
P Maroteaux, and
P Durand, and
J P Farriaux
August 2019,
Skeletal radiology,
