| N-terminal domain on dystroglycan enables LARGE1 to extend matriglycan on alpha-dystroglycan and prevents muscular dystrophy. | N-terminal domain on dystroglycan enables LARGE1 to extend matriglycan on α-dystroglycan and prevents muscular dystrophy.
Okuma H, Hord JM, Chandel I, Venzke D, Anderson ME, Walimbe AS, Joseph S, Gastel Z, Hara Y, Saito F, Matsumura K, Campbell KP., Free PMC Article | 02/23/2023 |
| GMPPA defects cause a neuromuscular disorder with alpha-dystroglycan hyperglycosylation. | GMPPA defects cause a neuromuscular disorder with α-dystroglycan hyperglycosylation.
Franzka P, Henze H, Jung MJ, Schüler SC, Mittag S, Biskup K, Liebmann L, Kentache T, Morales J, Martínez B, Katona I, Herrmann T, Huebner AK, Hennings JC, Groth S, Gresing L, Horstkorte R, Marquardt T, Weis J, Kaether C, Mutchinick OM, Ori A, Huber O, Blanchard V, von Maltzahn J, Hübner CA., Free PMC Article | 10/9/2021 |
| beta-dystroglycan intracellular domain acts as negative regulator of rDNA transcription by impeding the transcriptional activity of UBF, as a part of the protective mechanism activated in response to nucleolar stress | The intracellular domain of β-dystroglycan mediates the nucleolar stress response by suppressing UBF transcriptional activity.
Azuara-Medina PM, Sandoval-Duarte AM, Morales-Lázaro SL, Modragón-González R, Vélez-Aguilera G, Gómez-López JD, Jiménez-Gutiérrez GE, Tiburcio-Félix R, Martínez-Vieyra I, Suárez-Sánchez R, Längst G, Magaña JJ, Winder SJ, Ortega A, Ramos Perlingeiro RC, Jacobs LA, Cisneros B., Free PMC Article | 04/25/2020 |
| These findings establish Dystroglycan as a multifunctional scaffold that coordinates extracellular matrix proteins, secreted cues, and transmembrane receptors to regulate axon guidance. | Dystroglycan is a scaffold for extracellular axon guidance decisions.
Lindenmaier LB, Parmentier N, Guo C, Tissir F, Wright KM., Free PMC Article | 04/11/2020 |
| Our data suggest a mechanism where dystroglycan-mediated signaling between the cell and the extracellular matrix controls the proliferative potential of progenitors in the developing retina | Laminin β2 Chain Regulates Retinal Progenitor Cell Mitotic Spindle Orientation via Dystroglycan.
Serjanov D, Bachay G, Hunter DD, Brunken WJ., Free PMC Article | 11/2/2019 |
| alpha-DG hypoglycosylation, together with an increased protein instability, reduces the membrane dynamics of the beta-subunit and its clustering within the actin-rich domains, influencing cell migration and spontaneous cell movement. | α-Dystroglycan hypoglycosylation affects cell migration by influencing β-dystroglycan membrane clustering and filopodia length: A multiscale confocal microscopy analysis.
Palmieri V, Bozzi M, Signorino G, Papi M, De Spirito M, Brancaccio A, Maulucci G, Sciandra F. | 12/1/2018 |
| Utrn KO mdx mice but not mdx mice show decreased expression of serum N-terminal alpha Dystroglycan compared to normal mice. | N-terminal α Dystroglycan (αDG-N): A Potential Serum Biomarker for Duchenne Muscular Dystrophy.
Crowe KE, Shao G, Flanigan KM, Martin PT., Free PMC Article | 03/17/2018 |
| Loss of dystroglycan glycosylation is associated with dystroglycanopathies leading to a breakdown of muscle cell membrane integrity and eventual degeneration. | Distinct expression of functionally glycosylated alpha-dystroglycan in muscle and non-muscle tissues of FKRP mutant mice.
Blaeser A, Awano H, Lu P, Lu QL., Free PMC Article | 02/17/2018 |
| These results reveal a role for DG in maintaining the stability of the sarcomeric cytoskeleton during contraction. | Role of dystroglycan in limiting contraction-induced injury to the sarcomeric cytoskeleton of mature skeletal muscle.
Rader EP, Turk R, Willer T, Beltrán D, Inamori K, Peterson TA, Engle J, Prouty S, Matsumura K, Saito F, Anderson ME, Campbell KP., Free PMC Article | 01/27/2018 |
| Integrin beta1 and dystroglycan compensate each other to mediate laminin-dependent basement membrane assembly and epiblast polarization. | Integrin and dystroglycan compensate each other to mediate laminin-dependent basement membrane assembly and epiblast polarization.
Li S, Qi Y, McKee K, Liu J, Hsu J, Yurchenco PD., Free PMC Article | 01/6/2018 |
| Cleavage of beta-DG still occurred when both MMP-2 and MMP-9 were knocked out in gamma - sarcoglycan-deficient mice. The study found that up-regulation of MMP-14 is capable of cleaving beta-DG, and it may be involved in the pathogenesis of sarcoglycanopathy. | Cleavage of β-dystroglycan occurs in sarcoglycan-deficient skeletal muscle without MMP-2 and MMP-9.
Fukai Y, Ohsawa Y, Ohtsubo H, Nishimatsu SI, Hagiwara H, Noda M, Sasaoka T, Murakami T, Sunada Y. | 09/30/2017 |
| The extracellular environment plays a critical role in coordinating neuronal migration and neurite outgrowth during neural circuit development. | Dystroglycan Maintains Inner Limiting Membrane Integrity to Coordinate Retinal Development.
Clements R, Turk R, Campbell KP, Wright KM., Free PMC Article | 09/30/2017 |
| we provide evidence that a subset of GABAergic interneurons requires dystroglycan for formation and maintenance of axonal terminals on pyramidal cells | Neuronal Dystroglycan Is Necessary for Formation and Maintenance of Functional CCK-Positive Basket Cell Terminals on Pyramidal Cells.
Früh S, Romanos J, Panzanelli P, Bürgisser D, Tyagarajan SK, Campbell KP, Santello M, Fritschy JM., Free PMC Article | 07/29/2017 |
| These findings reveal a role for dystroglycan in orchestrating both the assembly and function of the subventricular zone neural stem cell niche | Dystroglycan Suppresses Notch to Regulate Stem Cell Niche Structure and Function in the Developing Postnatal Subventricular Zone.
McClenahan FK, Sharma H, Shan X, Eyermann C, Colognato H., Free PMC Article | 06/24/2017 |
| Fktn deficient mice express moderate to severe muscular dystrophy; glycosylated alpha-dystroglycan has a unique role in muscle regeneration in these mice | Abnormal Skeletal Muscle Regeneration plus Mild Alterations in Mature Fiber Type Specification in Fktn-Deficient Dystroglycanopathy Muscular Dystrophy Mice.
Foltz SJ, Modi JN, Melick GA, Abousaud MI, Luan J, Fortunato MJ, Beedle AM., Free PMC Article | 07/30/2016 |
| A glucuronic acid beta1,4-xylose disaccharide synthesized by B4GAT1 acts as an acceptor primer that can be elongated by LARGE with the ligand-binding heteropolysaccharide. | The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated α-dystroglycan functional glycosylation.
Willer T, Inamori K, Venzke D, Harvey C, Morgensen G, Hara Y, Beltrán Valero de Bernabé D, Yu L, Wright KM, Campbell KP., Free PMC Article | 04/16/2016 |
| This study demonstrated that the Postnatal development of the molecular complex underlying astrocyte polarization. | Postnatal development of the molecular complex underlying astrocyte polarization.
Lunde LK, Camassa LM, Hoddevik EH, Khan FH, Ottersen OP, Boldt HB, Amiry-Moghaddam M., Free PMC Article | 03/19/2016 |
| The X-ray crystal structure of the missense variant T190M of the murine N-terminal domain of alpha-dystroglycan (50-313) has been determined. | The Structure of the T190M Mutant of Murine α-Dystroglycan at High Resolution: Insight into the Molecular Basis of a Primary Dystroglycanopathy.
Bozzi M, Cassetta A, Covaceuszach S, Bigotti MG, Bannister S, Hübner W, Sciandra F, Lamba D, Brancaccio A., Free PMC Article | 01/30/2016 |
| 5-amino-4-imidazolecarboxamide riboside treatment increases utrophin A and beta-dystroglycan expression in mdx mouse muscle. | Utrophin A is essential in mediating the functional adaptations of mdx mouse muscle following chronic AMPK activation.
Al-Rewashdy H, Ljubicic V, Lin W, Renaud JM, Jasmin BJ. | 11/21/2015 |
| Phosphorylation within the cysteine-rich region of dystrophin enhances its association with beta-dystroglycan and identifies a potential novel therapeutic target for skeletal muscle wasting. | Phosphorylation within the cysteine-rich region of dystrophin enhances its association with β-dystroglycan and identifies a potential novel therapeutic target for skeletal muscle wasting.
Swiderski K, Shaffer SA, Gallis B, Odom GL, Arnett AL, Scott Edgar J, Baum DM, Chee A, Naim T, Gregorevic P, Murphy KT, Moody J, Goodlett DR, Lynch GS, Chamberlain JS., Free PMC Article | 07/25/2015 |
| Results suggest that by interfering in the cross-talk between the transmembrane form of the laminin receptor dystroglycan and F-actin, AHNAK1 influences the cytoskeleton organization of Schwann cells | Giant scaffolding protein AHNAK1 interacts with β-dystroglycan and controls motility and mechanical properties of Schwann cells.
von Boxberg Y, Soares S, Féréol S, Fodil R, Bartolami S, Taxi J, Tricaud N, Nothias F. | 03/21/2015 |
| We evaluate the key characteristics of the mdx in comparison with other mouse mutants with inactivations in DAPC components, along with key modifiers of the disease phenotype. | What do mouse models of muscular dystrophy tell us about the DAPC and its components?
Whitmore C, Morgan J., Free PMC Article | 03/7/2015 |
| results indicate a novel role for laminin-dystroglycan interactions in the cooperative integration of astrocytes, endothelial cells, and pericytes in regulating the Blood Brain Barrier. | The extracellular matrix protein laminin α2 regulates the maturation and function of the blood-brain barrier.
Menezes MJ, McClenahan FK, Leiton CV, Aranmolate A, Shan X, Colognato H., Free PMC Article | 02/14/2015 |
| Endogenous glucuronyltransferase activity of LARGE or LARGE2 required for functional modification of alpha-dystroglycan in cells and tissues. | Endogenous glucuronyltransferase activity of LARGE or LARGE2 required for functional modification of α-dystroglycan in cells and tissues.
Inamori K, Willer T, Hara Y, Venzke D, Anderson ME, Clarke NF, Guicheney P, Bönnemann CG, Moore SA, Campbell KP., Free PMC Article | 02/7/2015 |
| Beta-dystroglycan can respond to ezrin driven cytoskeletal and cell morphology changes,by translocating from the cytoplasm to the nucleus. | Nuclear import of β-dystroglycan is facilitated by ezrin-mediated cytoskeleton reorganization.
Vásquez-Limeta A, Wagstaff KM, Ortega A, Crouch DH, Jans DA, Cisneros B., Free PMC Article | 01/17/2015 |