Glycoprotein Catabolism in Brain Tissue in the Lysosomal Enzyme Deficiency Diseases

Brunngraber, Eric G.; Davis, Leonard G.; Javaid, Javaid I.; Berra, B.

doi:10.1007/978-1-4684-7735-1_3

Cited by 4 publications

(2 citation statements)

References 51 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Sphingolipidoses are genetically determined diseases where the deficiency of a specific lysosomal glycohydrolase leads to the storage of well defined glycolipids in brain and/or in visceral organs (Tettamanti et al, 1977;Brunngraber et al, 1976;Berra and Brunngraber, 1977). These stored glycolipids are thought to be responsible for the clinical signs involving, in most of the cases, the central nervous system.…”

mentioning

confidence: 99%

Studies on glycoprotein metabolism in different types of sphingolipidosis

Gasperi

Rapelli

Lindi

et al. 1981

J of Inher Metab Disea

View full text Add to dashboard Cite

show abstract

mentioning

confidence: 99%

Studies on glycoprotein metabolism in different types of sphingolipidosis

Gasperi

Rapelli

Lindi

et al. 1981

J of Inher Metab Disea

View full text Add to dashboard Cite

show abstract

“…Several classes of non-lipid storage compounds have also been isolated from a number of human patients. These include partially degraded keratan sulphates (Suzuki etal., 1969;Tsay & Dawson, 1973;Calatroni, 1974) and oligosaccharides or glycopeptides that appear to derive from cellular glycoproteins (Brunngraber et al, 1976;Calatroni, 1974;Wolfe et al, 1974).…”

mentioning

confidence: 99%

Hepatic storage of oligosaccharides and glycolipids in a cat affected with GM1 gangliosidosis

Holmes¹,

O’Brien²

1978

Biochemical Journal

View full text Add to dashboard Cite

1. Glycopeptides and glycolipids were isolated from normal cat liver and liver from a cat affected with GM1 gangliosidosis. 2. Bio-Gel P-6 chromatography of the crude glycopeptide fractions demonstrated three major peaks of hexose-containing compounds that were greatly increased in the mutant liver sample; these peaks contained oligosaccharides that comprised over 2% of the liver wet weight. 3. Two of the major pathological oligosaccharides, GP5 and GP6, were purified by chromatography on charcoal/Celite and Sephadex G-25. Oligosaccharides GP5 and GP6 had apparent mol.wts. of 1800 +/- 200 and 1350+/-200 respectively, and contained galactose, mannose and N-acetylglucosamine in molar proportions of 2.0:3.1:4.1 (GP5) and 1.0:2.2:2.7 (GP6). Periodate oxidation studies demonstrated the presence of galactose in a non-reducing terminal position. 4. The neutral glycolipid fraction from the mutant cat liver has a 1.3-fold increase in hexose content accompanied by an increased concentration of asialo-(ganglioside GM1). 5. There was a 2-fold increase of gangliosides in the mutant cat liver compared with normal cats. Ganglioside GM1 and a compound tentatively identified as N-glycolloyl-(ganglioside GM1) were the major glycolipids accumulated.

show abstract

Glycoprotein Storage Diseases and the Mucopolysaccharidoses

Dawson

1979

Complex Carbohydrates of Nervous Tissue

View full text Add to dashboard Cite

Glycoprotein Catabolism in Brain Tissue in the Lysosomal Enzyme Deficiency Diseases

Cited by 4 publications

References 51 publications

Studies on glycoprotein metabolism in different types of sphingolipidosis

Studies on glycoprotein metabolism in different types of sphingolipidosis

Hepatic storage of oligosaccharides and glycolipids in a cat affected with GM1 gangliosidosis

Glycoprotein Storage Diseases and the Mucopolysaccharidoses

Contact Info

Product

Resources

About