What do mouse models of muscular dystrophy tell us about the DAPC and its components?

Altered acetylcholine release in the hippocampus of dystrophin-deficient mice

(2014) S.F. Parames et al.   NEUROSCIENCE

Dystrophin complex functions as a scaffold for signalling proteins

(2013) Bruno Constantin   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Dmdmdx/Largemyd: a new mouse model of neuromuscular diseases useful for studying physiopathological mechanisms and testing therapies

(2013) P. C. M. Martins et al.   Disease Models & Mechanisms

Newborn bloodspot screening for Duchenne Muscular Dystrophy: 21 years experience in Wales (UK)

(2013) Stuart J Moat et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

The mdx mouse model as a surrogate for Duchenne muscular dystrophy

(2013) Terence A. Partridge   FEBS Journal

N-Glycolylneuraminic acid deficiency worsens cardiac and skeletal muscle pathophysiology in α-sarcoglycan-deficient mice

(2013) Paul T Martin et al.   GLYCOBIOLOGY

Mouse models of fukutin-related protein mutations show a wide range of disease phenotypes

(2013) Anthony Blaeser et al.   HUMAN GENETICS

The transgenic expression of LARGE exacerbates the muscle phenotype of dystroglycanopathy mice

(2013) Charlotte Whitmore et al.   HUMAN MOLECULAR GENETICS

Characteristics of magnetic resonance imaging biomarkers in a natural history study of golden retriever muscular dystrophy

(2013) Zheng Fan et al.   NEUROMUSCULAR DISORDERS

Extracellular microRNAs are dynamic non-vesicular biomarkers of muscle turnover

(2013) Thomas C. Roberts et al.   NUCLEIC ACIDS RESEARCH

Distinctive Serum miRNA Profile in Mouse Models of Striated Muscular Pathologies

(2013) Nicolas Vignier et al.   PLoS One

Mutation Types and Aging Differently Affect Revertant Fiber Expansion in Dystrophic Mdx and Mdx52 Mice

(2013) Yusuke Echigoya et al.   PLoS One

Identification of New Dystroglycan Complexes in Skeletal Muscle

(2013) Eric K. Johnson et al.   PLoS One

Muscle-specific expression of LARGE restores neuromuscular transmission deficits in dystrophic LARGEmyd mice

(2012) Jessica D. Gumerson et al.   HUMAN MOLECULAR GENETICS

Dystrophin-compromised sarcoglycan- -knockout diaphragm requires full wild-type embryonic stem cell reconstitution for correction

(2012) J. M. Vitale et al.   JOURNAL OF CELL SCIENCE

Mouse fukutin deletion impairs dystroglycan processing and recapitulates muscular dystrophy

(2012) Aaron M. Beedle et al.   JOURNAL OF CLINICAL INVESTIGATION

Comparative Proteomic Profiling of Dystroglycan-Associated Proteins in Wild Type, mdx, and Galgt2 Transgenic Mouse Skeletal Muscle

(2012) Jung Hae Yoon et al.   JOURNAL OF PROTEOME RESEARCH

The Shortest Isoform of Dystrophin (Dp40) Interacts with a Group of Presynaptic Proteins to Form a Presumptive Novel Complex in the Mouse Brain

(2012) Takenori Tozawa et al.   MOLECULAR NEUROBIOLOGY

Gene and cell-mediated therapies for muscular dystrophy

(2012) Patryk Konieczny et al.   MUSCLE & NERVE

Expression Analysis in Multiple Muscle Groups and Serum Reveals Complexity in the MicroRNA Transcriptome of the mdx Mouse with Implications for Therapy

(2012) Thomas C Roberts et al.   Molecular Therapy-Nucleic Acids

Leaky ryanodine receptors in β-sarcoglycan deficient mice: a potential common defect in muscular dystrophy

(2012) Daniel C Andersson et al.   Skeletal Muscle

mdx5cv Mice Manifest More Severe Muscle Dysfunction and Diaphragm Force Deficits than Do mdx Mice

(2011) Nicholas Beastrom et al.   AMERICAN JOURNAL OF PATHOLOGY

Dystroglycanopathies: coming into focus

(2011) Caroline Godfrey et al.   CURRENT OPINION IN GENETICS & DEVELOPMENT

Combined deficiency of alpha and epsilon sarcoglycan disrupts the cardiac dystrophin complex

(2011) Alessio Lancioni et al.   HUMAN MOLECULAR GENETICS

Dystrophin deficiency exacerbates skeletal muscle pathology in dysferlin-null mice

(2011) Renzhi Han et al.   Skeletal Muscle

Regulation of skeletal muscle growth by the IGF1-Akt/PKB pathway: insights from genetic models

(2011) Stefano Schiaffino et al.   Skeletal Muscle

Aberrant repair and fibrosis development in skeletal muscle

(2011) Christopher J Mann et al.   Skeletal Muscle

Genetic Background Affects Properties of Satellite Cells and mdx Phenotypes

(2010) So-ichiro Fukada et al.   AMERICAN JOURNAL OF PATHOLOGY

Aquaporin-4 expression is severely reduced in human sarcoglycanopathies and dysferlinopathies

(2010) Stefania Assereto et al.   CELL CYCLE

Fukutin-related protein is essential for mouse muscle, brain and eye development and mutation recapitulates the wide clinical spectrums of dystroglycanopathies

(2010) Yiumo Michael Chan et al.   HUMAN MOLECULAR GENETICS

Conditional knockout of protein O-mannosyltransferase 2 reveals tissue-specific roles of O-mannosyl glycosylation in brain development

(2010) Huaiyu Hu et al.   JOURNAL OF COMPARATIVE NEUROLOGY

Physiological Characterization of Muscle Strength With Variable Levels of Dystrophin Restoration in mdx Mice Following Local Antisense Therapy

(2010) Paul S Sharp et al.   MOLECULAR THERAPY

Magnetic resonance imaging assessment of cardiac dysfunction in δ-sarcoglycan null mice

(2010) Janaka P. Wansapura et al.   NEUROMUSCULAR DISORDERS

Uniquely human evolution of sialic acid genetics and biology

(2010) A. Varki   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A Human-Specific Deletion in Mouse Cmah Increases Disease Severity in the mdx Model of Duchenne Muscular Dystrophy

(2010) K. Chandrasekharan et al.   Science Translational Medicine

Genetic Manipulation of Dysferlin Expression in Skeletal Muscle

(2009) Douglas P. Millay et al.   AMERICAN JOURNAL OF PATHOLOGY

Overexpression of Galgt2 Reduces Dystrophic Pathology in the Skeletal Muscles of Alpha Sarcoglycan-Deficient Mice

(2009) Rui Xu et al.   AMERICAN JOURNAL OF PATHOLOGY

The neurobiology of the dystrophin-associated glycoprotein complex

(2009) Adrian Waite et al.   ANNALS OF MEDICINE

Reduced expression of fukutin related protein in mice results in a model for fukutin related protein associated muscular dystrophies

(2009) M. R. Ackroyd et al.   BRAIN

Sub-physiological sarcoglycan expression contributes to compensatory muscle protection in mdx mice

(2009) Dejia Li et al.   HUMAN MOLECULAR GENETICS

Preclinical drug trials in themdxmouse: Assessment of reliable and sensitive outcome measures

(2009) Christopher F. Spurney et al.   MUSCLE & NERVE

Mammalian animal models for Duchenne muscular dystrophy

(2009) Raffaella Willmann et al.   NEUROMUSCULAR DISORDERS

Blastocyst Injection of Wild Type Embryonic Stem Cells Induces Global Corrections in Mdx Mice

(2009) Elizabeth Stillwell et al.   PLoS One

Immune-Mediated Mechanisms Potentially Regulate the Disease Time-Course of Duchenne Muscular Dystrophy and Provide Targets for Therapeutic Intervention

(2009) Nicholas P. Evans et al.   PM&R

Overexpression of Galgt2 in skeletal muscle prevents injury resulting from eccentric contractions in both mdx and wild-type mice

(2008) Paul T. Martin et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

A common disease-associated missense mutation in alpha-sarcoglycan fails to cause muscular dystrophy in mice

(2008) Kazuhiro Kobuke et al.   HUMAN MOLECULAR GENETICS

Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy

(2008) Motoi Kanagawa et al.   HUMAN MOLECULAR GENETICS

Shifts in macrophage phenotypes and macrophage competition for arginine metabolism affect the severity of muscle pathology in muscular dystrophy

(2008) S. A. Villalta et al.   HUMAN MOLECULAR GENETICS

Brain and Eye Malformations Resembling Walker-Warburg Syndrome Are Recapitulated in Mice by Dystroglycan Deletion in the Epiblast

(2008) J. S. Satz et al.   JOURNAL OF NEUROSCIENCE

Reduced proliferative activity of primary POMGnT1-null myoblasts in vitro

(2008) Yuko Miyagoe-Suzuki et al.   MECHANISMS OF DEVELOPMENT

Destabilization of the Dystrophin-Glycoprotein Complex without Functional Deficits in α-Dystrobrevin Null Muscle

(2008) Tina M. Bunnell et al.   PLoS One