α-dystroglycan
Exogenous expression of the glycosyltransferase LARGE1 restores α-dystroglycan matriglycan and laminin binding in rhabdomyosarcoma
... of dystroglycan function has been asso- ciated with human pathologies, including muscular dys- trophy with or without various degrees of brain and eye defects ...[1]. Dystroglycan is synthesized as a single ...
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TRAPPC11 and GOSR2 mutations associate with hypoglycosylation of α-dystroglycan and muscular dystrophy
... Background: Transport protein particle (TRAPP) is a supramolecular protein complex that functions in localizing proteins to the Golgi compartment. The TRAPPC11 subunit has been implicated in muscle disease by virtue of ...
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Fukuyama-type congenital muscular dystrophy and defective glycosylation of α-dystroglycan
... In 2001, Hayashi et al. reported that the immunoreactiv- ity of the antibody against the sugar-chain moiety of a - dystroglycan is severely reduced in the skeletal muscle of patients with FCMD, suggesting ...
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Differences in Affinity of Binding of Lymphocytic Choriomeningitis Virus Strains to the Cellular Receptor α-Dystroglycan Correlate with Viral Tropism and Disease Kinetics
... ␣ -Dystroglycan ( ␣ -DG) was recently identified as a receptor for lymphocytic choriomeningitis virus (LCMV) and several other arenaviruses, including Lassa fever virus (W. Cao, M. D. Henry, P. Borrow, H. Yamada, ...
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New World Arenavirus Clade C, but Not Clade A and B Viruses, Utilizes α-Dystroglycan as Its Major Receptor
... ␣ -Dystroglycan ( ␣ -DG) has been identified as a major receptor for lymphocytic choriomeningitis virus (LCMV) and Lassa virus, two Old World arenaviruses. The situation with New World arenaviruses is less clear: ...
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Regulation of Laminin 1-Induced Pancreatic β-Cell Differentiation by α6 Integrin and α-Dystroglycan
... To investigate the role of 6 integrin in laminin- 1–induced -cell differentiation, pancreas cells were cultured with laminin-1 and the rat monoclonal an- tibody GoH3, which specifica[r] ...
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Posttranslational Modification of α-Dystroglycan, the Cellular Receptor for Arenaviruses, by the Glycosyltransferase LARGE Is Critical for Virus Binding
... To address the role of LARGE-dependent modification for the recognition of ␣-DG by LCMV isolates with high or low binding affinity, we examined the LCMV prototypic nonimmunosuppressive p[r] ...
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Characterization of the Interaction of Lassa Fever Virus with Its Cellular Receptor α-Dystroglycan
... The cellular receptor for the Old World arenaviruses Lassa fever virus (LFV) and lymphocytic choriomen- ingitis virus (LCMV) has recently been identified as ␣ -dystroglycan ( ␣ -DG), a cell surface receptor that ...
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O Mannosylation of α-Dystroglycan Is Essential for Lymphocytic Choriomeningitis Virus Receptor Function
... ␣ -Dystroglycan ( ␣ -DG) was identified as a common receptor for lymphocytic choriomeningitis virus (LCMV) and several other arenaviruses including the human pathogenic Lassa fever virus. Initial work postulated ...
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ISPD mutations account for a small proportion of Italian Limb Girdle Muscular Dystrophy cases
... coding for putative or demonstrated glycosyltransferase, have been associated with muscular dystrophies (referred to as secondary dystroglycanopathies) [19], while only a couple of cases have been associated with ...
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An evaluation of the evolution of the gene structure of dystroglycan
... of α-DG after the IG2 domain and the Gly-Ser site of proteolysis; a natively unfolded domain within the ectodomain of β-DG (NU); a single transmembrane domain and a cytoplasmic region that includes the ...
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Prenatal muscle development in a mouse model for the secondary dystroglycanopathies
... in α- dystroglycan glycosylation and laminin binding in the FKRP KD compared to wild ...in α-dystroglycan glycosylation are associated with an early reduction in the number of Pax7 + ...
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Dystroglycan Depletion Impairs Actin-Dependent Functions of Differentiated Kasumi-1 Cells
... of α-Dg varies from 120 to 180 kDa, depending on the extensive glyco- slylation, ...weight α-DG species [14], we determined the expression of a prominent band of 80 kDa as well as bands of 70 kDa and 60 kDa ...
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Congenital protein hypoglycosylation diseases
... of α -dystroglycan, a component of the dystrophin-glycoprotein (DAG) complex, has been impli- cated in several subtypes of muscular dystrophy, known as the ...of α -dystroglycan also ...
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Mouse fukutin deletion impairs dystroglycan processing and recapitulates muscular dystrophy
... on α-dystroglycan causes an array of congenital and limb girdle muscular dystrophies known as ...of dystroglycan in development has hampered elucidation of the mechanisms underlying ...glycosylated ...
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Retrograde trafficking of β-dystroglycan from the plasma membrane to the nucleus
... calnexin, an ER-resident protein; interestingly, β -DG (43 kDa) was recovered in the same calnexin-containing fractions (Fig. 4A). We analyzed the presence of early endosome (EEA1), cytoplasmic (GAPDH), PM (bioti- ...
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Milder forms of muscular dystrophy associated with POMGNT2 mutations
... that a reduction, rather than the complete loss, of the enzymatic activities due to missense mutations in FKTN and POMGNT2 may generate intermediate glycoforms of a-dystroglycan. However, it is impor- tant that ...
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TIM-1 Mediates Dystroglycan-Independent Entry of Lassa Virus
... ABSTRACT Lassa virus (LASV) is an Old World arenavirus responsible for hundreds of thousands of infections in West Africa every year. LASV entry into a variety of cell types is mediated by interactions with ...
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Podocin and Beta Dystroglycan expression to study Podocyte-Podocyte and basement membrane matrix connections in adult protienuric states
... beta dystroglycan involved in podocin-podocin interaction and podocyte - basement membrane and in cases of both primary and secondary podocytopathies (immune complex ...
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The 220th ENMC workshop: Dystroglycan and the Dystroglycanopathies held on the 27-29 May 2016, Naarden, The Netherlands
... Monika Liljedahl (mother of a boy with Limb Girdle Muscular Dystrophy and representative for Cure CMD) described how her son and his brother were IVF babies of parents 40 years of age. The pregnancy was uncomplicated and ...
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