Dystroglycans (Dystroglycan)

Dystrophin-associated proteins that play role in the formation of a transmembrane link between laminin-2 and DYSTROPHIN. Both the alpha and the beta subtypes of dystroglycan originate via POST-TRANSLATIONAL PROTEIN PROCESSING of a single precursor protein.

Also Known As:

Networked: 453 relevant articles (6 outcomes, 37 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Campbell, Kevin P: 42 articles (12/2015 - 07/2002)
2.	Muntoni, Francesco: 28 articles (04/2014 - 11/2002)
3.	Toda, Tatsushi: 24 articles (01/2015 - 03/2002)
4.	Moore, Steven A: 18 articles (12/2015 - 07/2002)
5.	Brancaccio, Andrea: 18 articles (01/2015 - 01/2003)
6.	Endo, Tamao: 17 articles (01/2015 - 03/2002)
7.	Kanagawa, Motoi: 17 articles (01/2015 - 07/2002)
8.	Torelli, Silvia: 16 articles (07/2013 - 11/2002)
9.	Willer, Tobias: 15 articles (12/2015 - 04/2005)
10.	Brown, Susan C: 15 articles (01/2015 - 11/2002)

Related Diseases

1.	Muscular Dystrophies (Muscular Dystrophy) 03/20/2006 - "We suggest that LNalpha1 chain in part ameliorates the development of LNalpha2 chain deficient muscular dystrophy by retaining the binding sites for integrin alpha7Bbeta1D and alpha-dystroglycan, respectively." 04/01/2013 - "To perform a proof-of-concept study, we selected four patients with congenital muscular dystrophies with defective glycosylation of alpha-dystroglycan. " 08/01/2010 - "This study was aimed to identify Fukutin (FKTN)-related congenital muscular dystrophies (CMD) with defective alpha-dystroglycan glycosylation in Korea and to discuss their genotype-phenotype spectrum focusing on detailed brain magnetic resonance imaging (MRI) findings. " 03/01/2010 - "Recent studies highlighted an emerging possibility of using Drosophila as a model system for investigating the mechanisms of human congenital muscular dystrophies, called dystroglycanopathies, resulting from the abnormal glycosylation of alpha-dystroglycan. " 05/26/2009 - "Congenital muscular dystrophies with defective glycosylation of dystroglycan: a population study."
2.	Duchenne Muscular Dystrophy (Muscular Dystrophy, Becker) 08/01/2004 - "These studies reveal novel functions and additional signalling roles for dystroglycan, raising the possibility of new avenues for therapeutic intervention in diseases such as Duchenne muscular dystrophy." 01/01/2015 - "DAG-6F4 immunolabelling was markedly reduced in muscle biopsies from Duchenne muscular dystrophy patients consistent with disruption of the dystroglycan complex. " 12/01/2005 - "Duchenne muscular dystrophy: alpha-dystroglycan immunoexpression in skeletal muscle and cognitive performance." 08/10/1998 - "Decreased expression of brain beta-dystroglycan in Duchenne muscular dystrophy but not in the mdx animal model." 06/01/2007 - "Overexpression of Galgt2, a glycosyltransferase not implicated in CMD, also alters dystroglycan glycosylation and inhibits muscular dystrophy in a mouse model of Duchenne muscular dystrophy. "
3.	Brain Edema (Cerebral Edema) 02/19/2015 - "Loss of AQP4 polarized localization with loss of β-dystroglycan immunoreactivity may induce brain edema following intracerebral hemorrhage." 01/01/2015 - "To understand how aquaporin4 (AQP4) and dystroglycan (DG) polarized distribution change and their roles in brain edema formation after traumatic brain injury (TBI). " 01/01/2011 - "Indeed, mice lacking components of this complex or in which laminin-dystroglycan interaction is disrupted show a delayed onset of brain edema due to a redistribution of AQP4 away from astrocyte endfeet. " 01/01/2011 - "These findings reveal the mechanism of action of chloranil in preventing the laminin-induced clustering of dystroglycan and AQP4 and validate the use of high-throughput screening as a tool to identify drugs that modulate AQP4 clustering and that could be tested in models of brain edema."
4.	Alveolar Rhabdomyosarcoma 11/01/2007 - "Using a combination of immunostaining on tissue microarrays and immunoblotting of snap-frozen unfixed tissues, we demonstrate a significant reduction in native alpha dystroglycan expression in pediatric alveolar rhabdomyosarcoma (RMS), embryonal RMS, neuroblastoma (NBL), and medulloblastoma, whereas expression of beta dystroglycan, which is cotranslated with alpha dystroglycan, is largely unchanged. "
5.	Neuroblastoma 11/01/2007 - "Neuroblastoma and RMS samples with reduced expression of native alpha dystroglycan also showed reduced laminin binding in laminin overlay experiments. " 11/01/2007 - "Using a combination of immunostaining on tissue microarrays and immunoblotting of snap-frozen unfixed tissues, we demonstrate a significant reduction in native alpha dystroglycan expression in pediatric alveolar rhabdomyosarcoma (RMS), embryonal RMS, neuroblastoma (NBL), and medulloblastoma, whereas expression of beta dystroglycan, which is cotranslated with alpha dystroglycan, is largely unchanged. "

Related Drugs and Biologics

1.	Dystrophin
2.	Laminin (Merosin)
3.	Dystroglycans (Dystroglycan)
4.	Glycoproteins (Glycoprotein)
5.	Proteins (Proteins, Gene)
6.	Carrier Proteins (Binding Protein)
7.	Sarcoglycans (beta Sarcoglycan)
8.	Agrin
9.	Transferases
10.	Membrane Proteins (Integral Membrane Proteins)

Related Therapies and Procedures

1.	Tenotomy
2.	Traction