> > , .2
<<
>>
<< >>

. , , .

, , . , IdUA, , , , GlcUA .

, , () (), . IdUA , , . 2 5 . ( I, II 111) .

.

, . 100 .

, , , . . , , -, , . , .

, . ( IdUA ) . IX XI. - , III. (1:1) III , . Lys III, -, , . . 54.13.

2 , , , III. 10 , , . - IdUA- .

, , III, .

-, , . , , . - , III.

. 54.13. (, ), . , -, , , . ( .) . (Reproduced, with permission, from Lennarz W.J. The Biochemistry of Glycoproteins and Proteoglycans. Plenum Press, 1980.)

, , . , , . .

, . , , -, , .

, , , , . , .

, , . , - in vitro. , . , , .

, . .

, , , , , . - , , .

Buckwalter J. A., Rosenberg L. . Electron microscopic studies of cartilage proteoglycans, J. Biol. Chem., 1982, 257, 9830. DiNatale P., Neufeld E. F. The biochemical diagnosis of mucopolysaccharidoses, mucolipidosis and related disorders. In: Perspectives in Inherited Metabolic Diseases, Vol. 2, Barra B. et al. (eds.), Editiones Ermes (Milan), 1979.

Hook M. et al. Cell-surface glycosaminoglycans, Annu. Rev.

Biochem., 1984, 53, 847.

Hubbard S. C., Ivatt R. J. Synthesis and processing of asparagine-linked oligosaccharides, Annu. Rev. Biochem., 1981, 50, 555.

Jagues L. B. Heparin: an old drug with a new paradigm. Science, 1979, 206, 528.

Kornfeld R., Kornfeld S. Assembly of asparagine-linked oligosaccharides, Annu. Rev. Biochem., 1985, 54, 631. Kornfeld S., Sly W. S. Lysosomal storage defects, Hosp. Pract. (Aug), 1985, 20, 71.

Lennarz W. J. The Biochemistry of Glycoproteins and Proteoglycans, Plenum Press, 1980.

Poole A. R. et al. Proteaglycans from bovine nasal cartilage: Immunochemical studies of link protein, J. Biol. Chem., 1980, 255, 9295.

Reitman M. L. et al. Fibroblasts from patients with I-cell disease and pseudo-Hurler polydystrophy are deficient in uridine 5-diphosphate-N-acetylglucosamine: glycoprotein N-acetylglucosaminylphosphotranspherase activity, J. Clin. Invest., 1981, 67, 1574.

Schachter H. Biosynthetic controls that determine the branching and heterogeneity of protein-bound oligosaccharides, Biochem. Cell Biol., 1986, 64, 163.

Snider M. D., Rogers . C. Transmembrane movement of oligo-saccharide-lipids during glycoprotein synthesis, Cell, 1984, 36, 753.

<< >>