Structure of the glycopeptide storage material in G M1 gangliosidosis Sequence determination with specific endo- and exoglycosidases

1. 1. An endo-β-galactosidase from Escherichia freundii, specific for the hydrolysis of desulfated keratan sulfate, quantitatively liberated a trisaccharide (GalGlcNAcGal) from a glycopeptide ( M r 1800) isolated from the liver of a patient with G M 1 (generalized) gangliosidosis. 2. 2. The remain...

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Published inBiochimica et biophysica acta. General subjects Vol. 385; no. 2; pp. 305 - 311
Main Authors Chen Tsay, Grace, Dawson, Glyn, Li, Yu-Teh
Format Journal Article
LanguageEnglish
Published Elsevier B.V 1975
Subjects
Ac
G M1
AcNeu
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ISSN0304-4165
1872-8006
DOI10.1016/0304-4165(75)90358-X

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Summary:1. 1. An endo-β-galactosidase from Escherichia freundii, specific for the hydrolysis of desulfated keratan sulfate, quantitatively liberated a trisaccharide (GalGlcNAcGal) from a glycopeptide ( M r 1800) isolated from the liver of a patient with G M 1 (generalized) gangliosidosis. 2. 2. The remaining glycopeptide was susceptible to sequential digestion with purified β- N-acetylhexosaminidase and exo-β-galactosidase from Jack Bean meal. 3. 3. These and other studies established of the structure fo the stored glycopeptide to be: ▪ which probably represents the desulfated linkage region of skeletal keratan sulfate.
ISSN:0304-4165
1872-8006
DOI:10.1016/0304-4165(75)90358-X