Glycoproteins degradation

Scriver CR et al (editors) The Metabolic and Molecular Bases of In-heritedDisease, 8th ed. McGraw-Hill, 2001.(Various chapters in this text give in-depth coverage of topics such as I-cell disease and disorders of glycoprotein degradation.)... 

Schubert U, Anton LC, Badk I, Cox JH, Bour S, Bennink JR, Orlowski M, Strebel K, Yewdell JW (1998) CD4 glycoprotein degradation induced by human immunodeficiency virus type 1 Vpu protein requires the function of proteasomes and the ubiquitin-conjugating pathway. J Virol 72 2280-2288... 

Table 4.2.1 lists the disorders of glycoprotein degradation that are detected by urinary oligosaccharide screening. It is beyond the scope of this article to give clinical descriptions of all of the disorders listed therefore the reader is referred to the references given in Table 4.2.1. 

Thomas GH (2001) Disorders of glycoprotein degradation a-mannosidosis, / -mannosidosis, fucosidosis and sialidosis. In Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The Metabolic and Molecular Bases of Inherited Disease, vol III, 8th edn. McGraw-Hill, New York, pp 3507-3533... 

G.H. Thomas, Disorders of glycoprotein degradation and straeture A-mannosidosis, b-mannosidosis, fucosidosis, and siaUdosis, in C. R. Scriver, A. L. Beaudet, W. S. Sly, and D. Valle (Eds.), The Metabolic and Molecular Bases of Inherited Disease, McGraw-Hill Inc, New York, 2001, pp. 3507-3534. 

Romisch K, Ali HR (1997) Similar processes mediate glycopcptide export from the endoplasmic reticulum in mammalian cells and Saccharomyces cerevisiae. Proc Nall Acad Sci USA 94 6730- 6734 Romisch K, Schekman R (1992) Distinct processes mediate glycoprotein and glycopeptide export from the endoplasmic reticulum in Saccharomyces cerevisiae. Proc Natl Acad Sci USA 89 7227 7231 Schubert U, Anton LC, Bacik I, Cox JH, Bour S, Bennink JR, Orlowski M, Strebel K, Yewdell JW (1998) CD4 glycoprotein degradation induced by human immunodeficiency virus type I Vpu protein requires the function of proteasomes and the ubiquitin-conjugating pathway. J Virol 72 2280-2288... 

The oligosaccharidoses are a group of lysosomal storage disorders characterized by defects of glycoprotein degradation due to the deficiency of specific lysosomal enzymes (Fig. 19.1). All are inherited as autosomal recessive traits. 

Early Trimming Events importance for quality control glycoprotein degradation and anteriograde transport... 

Cantz, M. Ulrich-Bott, B. Disorders of Glycoprotein Degradation. J. Inherit. Metab. Dis. 1990, 13, 523-537. 

Figure 2 depicts schematically some of the typical carbohydrate chains of glycoproteins and the enzymes involved in each step of degradation. As the figure shows, there are two major ways to initiate glycoprotein degradation. It can be started either by a sequential hydrolysis of the saccharide portion or by degradation of the protein backbone by the action of a variety of proteolytic enzymes. In fact. 

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