Article

The ABCA1 cholesterol transporter associates with one of two distinct dystrophin-based scaffolds in Schwann cells.

Department of Physiology and Biophysics, University of Washington, 1959 NE Pacific St, Box 357290, Seattle WA 98195-7290, USA
Glia (impact factor: 4.82). 05/2008; 56(6):611-8. DOI:10.1002/glia.20636 pp.611-8
Source: PubMed

ABSTRACT Cytoskeletal scaffolding complexes help organize specialized membrane domains with unique functions on the surface of cells. In this study, we define the scaffolding potential of the Schwann cell dystrophin glycoprotein complex (DGC) by establishing the presence of four syntrophin isoforms, (alpha1, beta1, beta2, and gamma2), and one dystrobrevin isoform, (alpha-dystrobrevin-1), in the abaxonal membrane. Furthermore, we demonstrate the existence of two separate DGCs in Schwann cells that divide the abaxonal membrane into spatially distinct domains, the DRP2/periaxin rich plaques and the Cajal bands that contain Dp116, utrophin, alpha-dystrobrevin-1 and four syntrophin isoforms. Finally, we show that the two different DGCs can scaffold unique accessory molecules in distinct areas of the Schwann cell membrane. Specifically, the cholesterol transporter ABCA1, associates with the Dp116/syntrophin complex in Cajal bands and is excluded from the DRP2/periaxin rich plaques.

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    Article: Biological role of dystroglycan in Schwann cell function and its implications in peripheral nervous system diseases.
    Toshihiro Masaki, Kiichiro Matsumura
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    ABSTRACT: Dystroglycan is a central component of the dystrophin-glycoprotein complex (DGC) that links extracellular matrix with cytoskeleton, expressed in a variety of fetal and adult tissues. Dystroglycan plays diverse roles in development and homeostasis including basement membrane formation, epithelial morphogenesis, membrane stability, cell polarization, and cell migration. In this paper, we will focus on biological role of dystroglycan in Schwann cell function, especially myelination. First, we review the molecular architecture of DGC in Schwann cell abaxonal membrane. Then, we will review the loss-of-function studies using targeted mutagenesis, which have revealed biological functions of each component of DGC in Schwann cells. Based on these findings, roles of dystroglycan in Schwann cell function, in myelination in particular, and its implications in diseases will be discussed in detail. Finally, in view of the fact that understanding the role of dystroglycan in Schwann cells is just beginning, future perspectives will be discussed.
    Journal of Biomedicine and Biotechnology 01/2010; 2010:740403. · 2.44 Impact Factor
Data provided are for informational purposes only. Although carefully collected, accuracy cannot be guaranteed. The impact factor represents a rough estimation of the journal's impact factor and does not reflect the actual current impact factor. Publisher conditions are provided by RoMEO. Differing provisions from the publisher's actual policy or licence agreement may be applicable.

Keywords

alpha1
 
beta1
 
cholesterol transporter ABCA1
 
Cytoskeletal scaffolding complexes
 
distinct areas
 
Dp116/syntrophin complex
 
DRP2/periaxin rich plaques
 
gamma2
 
scaffolding potential
 
Schwann cell dystrophin glycoprotein complex
 
Schwann cell membrane
 
Schwann cells
 
separate DGCs
 
spatially distinct domains
 
specialized membrane domains
 
syntrophin isoforms
 
unique functions
 
utrophin