Abnormal expression of heparan sulfate proteoglycan on basal lamina of muscle fibers in two Japanese patients with adhalin deficiency

Hybridoma techniques have been used to generate monoclonal antibodies to an antigen concentrated in the basal lamina at the Xenopus laevis neuromuscular junction. The antibodies selectively precipitate a high molecular weight heparan sulfate proteoglycan from conditioned medium of muscle cultures grown in the presence of [35S]methionine or [35S]sulfate. Electron microscope autoradiography of adult X. laevis muscle fibers exposed to 125I-labeled antibody confirms that the antigen is localized within the basal lamina of skeletal muscle fibers and is concentrated at least fivefold within the specialized basal lamina at the neuromuscular junction. Fluorescence immunocytochemical experiments suggest that a similar proteoglycan is also present in other basement membranes, including those associated with blood vessels, myelinated axons, nerve sheath, and notochord. During development in culture, the surface of embryonic muscle cells displays a conspicuously non-uniform distribution of this basal lamina proteoglycan, consisting of large areas with a low antigen site-density and a variety of discrete plaques and fibrils. Clusters of acetylcholine receptors that form on muscle cells cultured without nerve are invariably associated with adjacent, congruent plaques containing basal lamina proteoglycan. This is also true for clusters of junctional receptors formed during synaptogenesis in vitro. This correlation indicates that the spatial organization of receptor and proteoglycan is coordinately regulated, and suggests that interactions between these two species may contribute to the localization of acetylcholine receptors at the neuromuscular junction.

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Basal Lamina Heparan Sulfate Proteoglycan, Perlecan, as a Low Affinity Receptor for Fibroblast Growth Factor.

Trends in Glycoscience and Glycotechnology ◽

10.4052/tigg.9.357 ◽

1997 ◽

Vol 9 (48) ◽

pp. 357-358

Author(s):

A. Yoneda

Keyword(s):

Growth Factor ◽

Heparan Sulfate ◽

Fibroblast Growth Factor ◽

Basal Lamina ◽

Heparan Sulfate Proteoglycan ◽

Sulfate Proteoglycan ◽

Fibroblast Growth ◽

Affinity Receptor

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Biosynthesis and Deposition of a Noncovalent Laminin-Heparan Sulfate Proteoglycan Complex and Other Basal Lamina Components by a Human Malignant Cell Line

Journal of Biological Chemistry ◽

10.1016/s0021-9258(18)94033-0 ◽

1989 ◽

Vol 264 (6) ◽

pp. 3078-3088

Author(s):

G P Frenette ◽

R W Ruddon ◽

R F Krzesicki ◽

J A Naser ◽

B P Peters

Keyword(s):

Heparan Sulfate ◽

Cell Line ◽

Basal Lamina ◽

Malignant Cell ◽

Heparan Sulfate Proteoglycan ◽

Sulfate Proteoglycan ◽

Malignant Cell Line

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Localization of type IV collagen, laminin, heparan sulfate proteoglycan, and fibronectin to the basal lamina of basement membranes.

The Journal of Cell Biology ◽

10.1083/jcb.95.1.340 ◽

1982 ◽

Vol 95 (1) ◽

pp. 340-344 ◽

Cited By ~ 322

Author(s):

G W Laurie ◽

C P Leblond ◽

G R Martin

Keyword(s):

Basement Membrane ◽

Heparan Sulfate ◽

Basal Lamina ◽

Type Iv Collagen ◽

Heparan Sulfate Proteoglycan ◽

Basement Membranes ◽

Enamel Organ ◽

Sulfate Proteoglycan ◽

Type Iv ◽

Membrane Region

Electron microscopic immunostaining of rat duodenum and incisor tooth was used to examine the location of four known components of the basement-membrane region: type IV collagen, laminin, heparan sulfate proteoglycan, and fibronectin. Antibodies or antisera against these substances were localized by direct or indirect peroxidase methods on 60-microns thick slices of formaldehyde-fixed tissues. In the basement-membrane region of the duodenal epithelium, enamel-organ epithelium, and blood-vessel endothelium, immunostaining for all four components was observed in the basal lamina (also called lamina densa). The bulk of the lamina lucida (rara) was unstained, but it was traversed by narrow projections of the basal lamina that were immunostained for all four components. In the subbasement-membrane fibrous elements or reticular lamina, immunostaining was confined to occasional "bridges" extending from the epithelial basal-lamina to that of adjacent capillaries. The joint presence of type IV collagen, laminin, heparan sulfate proteoglycan, and fibronectin in the basal lamina indicates that these substances do not occur in separate layers but are integrated into a common structure.

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