A-dystroglycan

The lepromatous form of leprosy is characterized by loss ofcutaneoussensibility. Hansen sbacillus(Mycobacterium leprae), which proliferates only in environments cooler than the core temperature maintained by most mammals, is capable of infecting Schwann cells in subcutaneous nerves because the basal lamina of these cells contains a-dystroglycan, to which this mycobacterium binds, and because subcutaneous nerves are often cooler than deeper tissues. Lepromatous neuropathy is a common cause of sensory mononeuropathy multiplex in the developing World [16,17]. 

Other human O-glycosylation deficiencies that are not detectable by IEF of ApoC-III affect oligosaccharides in O-mannosidic linkage to a-dystroglycan in Walker-Warburg syndrome and muscle-eye-brain disease [2, 32], and fucosylated N- and... 

While one end of the dystrophin molecule binds to actin filaments, the C-terminal domain associates with several additional proteins to form a dystrophin-glycoprotein complex (see figure)/1 k Dystrophin is linked directly to the membrane-spanning protein P-dystroglycan, which in the outer membrane surfaces associates with a glycoprotein a-dystroglycan. The latter binds to laminin-2 (Fig. 8-33), a protein that binds the cell to the basal lamina. Four... 

Hohenester, E., Tisi, D., Talts, J. F., and Timpl, R. (1999). The crystal structure of a laminin G-like module reveals the molecular basis of a-dystroglycan binding to laminins, perlecan, and agrin. Mol. Cell 4, 783-792. 

Chiba A, Matsumura K, Yamada H, Inazu.T, Shimizu T, Kusunoki S, Kanazawa 1, Kobata A, Endo T. Stmctures of sialylated O-linked oligosaccharides of bovine peripheral nerve a-dystroglycan the role of a novel O-mannosyl-type oligosaccharide in the binding of a-dystroglycan with laminin. J. Biol. Chem. 1997 272(4) 2156-2162. 

Seifert, J, Ogawa, T, Ito, Y, Syntheses of a-dystroglycan derived sialylated glycosyl amino acids carrying a novel mannosyl serine/threonine linkage. Tetrahedron Lett., 40, 6803-6807, 1999. 

Laminin-1 (laminin 111) Integrin (al(3l, a2 3l, a6 3l, a7 3l, a9pi), a-dystroglycan,suifade, and heparan sulfate proteoglycan... 

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