Published in FASEB J on November 25, 2013
Reactivation of autophagy by spermidine ameliorates the myopathic defects of collagen VI-null mice. Autophagy (2015) 0.81
The Role of Collagens in Peripheral Nerve Myelination and Function. Mol Neurobiol (2014) 0.80
Aggresome-Autophagy Involvement in a Sarcopenic Patient with Rigid Spine Syndrome and a p.C150R Mutation in FHL1 Gene. Front Aging Neurosci (2014) 0.80
Human Schwann cells seeded on a novel collagen-based microstructured nerve guide survive, proliferate, and modify neurite outgrowth. Biomed Res Int (2014) 0.78
Transcriptional profiles of supragranular-enriched genes associate with corticocortical network architecture in the human brain. Proc Natl Acad Sci U S A (2016) 0.77
Identification of candidate genes that underlie the QTL on chromosome 1 that mediates genetic differences in stress-ethanol interactions. Physiol Genomics (2015) 0.77
Lack of collagen VI promotes neurodegeneration by impairing autophagy and inducing apoptosis during aging. Aging (Albany NY) (2016) 0.75
Laminin targeting of a peripheral nerve-highlighting peptide enables degenerated nerve visualization. Proc Natl Acad Sci U S A (2016) 0.75
Adhesion G-Protein Coupled Receptors and Extracellular Matrix Proteins: Roles in Myelination and Glial Cell Development. Dev Dyn (2016) 0.75
Lack of Collagen VI Promotes Wound-Induced Hair Growth. J Invest Dermatol (2015) 0.75
Guidelines for the use and interpretation of assays for monitoring autophagy. Autophagy (2012) 20.08
Metabolic dysregulation and adipose tissue fibrosis: role of collagen VI. Mol Cell Biol (2008) 4.75
Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency. Nat Genet (2003) 3.15
Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration. Nat Med (2010) 3.01
Emilin1 links TGF-beta maturation to blood pressure homeostasis. Cell (2006) 2.84
Adipocyte-derived collagen VI affects early mammary tumor progression in vivo, demonstrating a critical interaction in the tumor/stroma microenvironment. J Clin Invest (2005) 2.38
Mitochondrial dysfunction in the pathogenesis of Ullrich congenital muscular dystrophy and prospective therapy with cyclosporins. Proc Natl Acad Sci U S A (2007) 1.74
Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies. Proc Natl Acad Sci U S A (2008) 1.59
Collagen VI regulates satellite cell self-renewal and muscle regeneration. Nat Commun (2013) 1.46
Physical exercise stimulates autophagy in normal skeletal muscles but is detrimental for collagen VI-deficient muscles. Autophagy (2011) 1.44
Three novel collagen VI chains with high homology to the alpha3 chain. J Biol Chem (2008) 1.42
EMILIN-1 deficiency induces elastogenesis and vascular cell defects. Mol Cell Biol (2004) 1.41
Developmental and osteoarthritic changes in Col6a1-knockout mice: biomechanics of type VI collagen in the cartilage pericellular matrix. Arthritis Rheum (2009) 1.36
Pericyte deficiencies lead to aberrant tumor vascularizaton in the brain of the NG2 null mouse. Dev Biol (2010) 1.34
Denervation in murine fast-twitch muscle: short-term physiological changes and temporal expression profiling. Physiol Genomics (2005) 1.27
Collagen VI protects neurons against Abeta toxicity. Nat Neurosci (2009) 1.19
Bethlem myopathy (BETHLEM) and Ullrich scleroatonic muscular dystrophy: 100th ENMC international workshop, 23-24 November 2001, Naarden, The Netherlands. Neuromuscul Disord (2002) 1.19
Genetic ablation of cyclophilin D rescues mitochondrial defects and prevents muscle apoptosis in collagen VI myopathic mice. Hum Mol Genet (2009) 1.17
Misregulation of autophagy and protein degradation systems in myopathies and muscular dystrophies. J Cell Sci (2013) 1.17
Oxidative stress by monoamine oxidases is causally involved in myofiber damage in muscular dystrophy. Hum Mol Genet (2010) 1.15
Dysfunction of mitochondria and sarcoplasmic reticulum in the pathogenesis of collagen VI muscular dystrophies. Ann N Y Acad Sci (2008) 1.15
Drosophila CAKI/CMG protein, a homolog of human CASK, is essential for regulation of neurotransmitter vesicle release. J Neurophysiol (2005) 1.08
Autophagy induction rescues muscular dystrophy. Autophagy (2011) 1.08
Skeletal muscle, autophagy, and physical activity: the ménage à trois of metabolic regulation in health and disease. J Mol Med (Berl) (2013) 1.07
Dysfunctional tendon collagen fibrillogenesis in collagen VI null mice. Matrix Biol (2010) 1.06
Absence of type VI collagen paradoxically improves cardiac function, structure, and remodeling after myocardial infarction. Circ Res (2012) 1.05
Cationic PMMA nanoparticles bind and deliver antisense oligoribonucleotides allowing restoration of dystrophin expression in the mdx mouse. Mol Ther (2009) 1.04
Contributions of adipose tissue architectural and tensile properties toward defining healthy and unhealthy obesity. Am J Physiol Endocrinol Metab (2013) 1.02
Collagen VI in cancer and its biological mechanisms. Trends Mol Med (2013) 1.01
Overlapping, complementary and site-specific expression pattern of genes of the EMILIN/Multimerin family. Matrix Biol (2004) 1.00
Expression of collagen VI α5 and α6 chains in human muscle and in Duchenne muscular dystrophy-related muscle fibrosis. Matrix Biol (2011) 0.99
Differential and restricted expression of novel collagen VI chains in mouse. Matrix Biol (2011) 0.97
Persistent dystrophin protein restoration 90 days after a course of intraperitoneally administered naked 2'OMePS AON and ZM2 NP-AON complexes in mdx mice. J Biomed Biotechnol (2012) 0.97
Alternative S2 hinge regions of the myosin rod differentially affect muscle function, myofibril dimensions and myosin tail length. J Mol Biol (2007) 0.95
Identification of a deep intronic mutation in the COL6A2 gene by a novel custom oligonucleotide CGH array designed to explore allelic and genetic heterogeneity in collagen VI-related myopathies. BMC Med Genet (2010) 0.95
Post-transcriptional silencing and functional characterization of the Drosophila melanogaster homolog of human Surf1. Genetics (2005) 0.95
Role of macrophage polarization in tumor angiogenesis and vessel normalization: implications for new anticancer therapies. Int Rev Cell Mol Biol (2013) 0.95
Collagen VI myopathies: from the animal model to the clinical trial. Adv Enzyme Regul (2009) 0.94
A Drosophila mutant of LETM1, a candidate gene for seizures in Wolf-Hirschhorn syndrome. Hum Mol Genet (2009) 0.93
Collagen VI ablation retards brain tumor progression due to deficits in assembly of the vascular basal lamina. Am J Pathol (2011) 0.93
Sphingosine 1-phosphate protects mouse extensor digitorum longus skeletal muscle during fatigue. Am J Physiol Cell Physiol (2005) 0.93
Expression of the collagen VI α5 and α6 chains in normal human skin and in skin of patients with collagen VI-related myopathies. J Invest Dermatol (2010) 0.93
Identification and characterization of novel collagen VI non-canonical splicing mutations causing Ullrich congenital muscular dystrophy. Hum Mutat (2009) 0.92
Altered expression of the MCSP/NG2 chondroitin sulfate proteoglycan in collagen VI deficiency. Mol Cell Neurosci (2005) 0.92
Cyclosporine A in Ullrich congenital muscular dystrophy: long-term results. Oxid Med Cell Longev (2011) 0.92
Post-transcriptional silencing of the Drosophila homolog of human ZASP: a molecular and functional analysis. Cell Tissue Res (2009) 0.91
Altered threshold of the mitochondrial permeability transition pore in Ullrich congenital muscular dystrophy. Biochim Biophys Acta (2008) 0.90
Altered trabecular bone structure and delayed cartilage degeneration in the knees of collagen VI null mice. PLoS One (2012) 0.90
Contractile properties and myosin heavy chain isoform composition in single fibre of human laryngeal muscles. J Muscle Res Cell Motil (2002) 0.90
Autophagy is Impaired in the Tibialis Anterior of Dystrophin Null Mice. PLoS Curr (2013) 0.88
Mitochondrial dysfunction and defective autophagy in the pathogenesis of collagen VI muscular dystrophies. Cold Spring Harb Perspect Biol (2013) 0.87
An enhancer required for transcription of the Col6a1 gene in muscle connective tissue is induced by signals released from muscle cells. Exp Cell Res (2008) 0.87
Antisense-induced messenger depletion corrects a COL6A2 dominant mutation in Ullrich myopathy. Hum Gene Ther (2012) 0.86
Expression of the EMILIN-1 gene during mouse development. Matrix Biol (2002) 0.86
From action potential to contraction: neural control and excitation-contraction coupling in larval muscles of Drosophila. Comp Biochem Physiol A Mol Integr Physiol (2009) 0.85
Type VI Collagen Regulates Pericellular Matrix Properties, Chondrocyte Swelling, and Mechanotransduction in Mouse Articular Cartilage. Arthritis Rheumatol (2015) 0.84
Muscle proteomics reveals novel insights into the pathophysiological mechanisms of collagen VI myopathies. J Proteome Res (2014) 0.84
On the pathogenesis of collagen VI muscular dystrophies--comment on article of Hicks et al. Brain (2009) 0.84
EMILIN-3, peculiar member of elastin microfibril interface-located protein (EMILIN) family, has distinct expression pattern, forms oligomeric assemblies, and serves as transforming growth factor β (TGF-β) antagonist. J Biol Chem (2012) 0.84
Emilin genes are duplicated and dynamically expressed during zebrafish embryonic development. Dev Dyn (2008) 0.83
Ultrastructural defects of collagen VI filaments in an Ullrich syndrome patient with loss of the alpha3(VI) N10-N7 domains. J Cell Physiol (2006) 0.83
Collagen VI regulates peripheral nerve regeneration by modulating macrophage recruitment and polarization. Acta Neuropathol (2014) 0.83
The notochord: structure and functions. Cell Mol Life Sci (2015) 0.83
Lentiviral-mediated RNAi in vivo silencing of Col6a1, a gene with complex tissue specific expression pattern. J Biotechnol (2009) 0.82
Transition of Homer isoforms during skeletal muscle regeneration. Am J Physiol Cell Physiol (2005) 0.82
Early changes of type 2B fibers after denervation of rat EDL skeletal muscle. J Appl Physiol (1985) (2002) 0.82
Annexin A2 mediates secretion of collagen VI, pulmonary elasticity and apoptosis of bronchial epithelial cells. J Cell Sci (2013) 0.82
Human adipose-derived stem cell transplantation as a potential therapy for collagen VI-related congenital muscular dystrophy. Stem Cell Res Ther (2014) 0.82
Type VI collagen deficiency induces osteopenia with distortion of osteoblastic cell morphology. Tissue Cell (2011) 0.82
Defhc1.1, a homologue of the juvenile myoclonic gene EFHC1, modulates architecture and basal activity of the neuromuscular junction in Drosophila. Hum Mol Genet (2011) 0.80
Synaptic activity modifies the levels of Dorsal and Cactus at the neuromuscular junction of Drosophila. J Neurobiol (2003) 0.80
The Role of Collagens in Peripheral Nerve Myelination and Function. Mol Neurobiol (2014) 0.80
S-nitrosoglutathione reductase deficiency-induced S-nitrosylation results in neuromuscular dysfunction. Antioxid Redox Signal (2014) 0.79
EMILIN2 down-modulates the Wnt signalling pathway and suppresses breast cancer cell growth and migration. J Pathol (2014) 0.79
Changes in muscle cell metabolism and mechanotransduction are associated with myopathic phenotype in a mouse model of collagen VI deficiency. PLoS One (2013) 0.78
Analysis of regulatory regions of Emilin1 gene and their combinatorial contribution to tissue-specific transcription. J Biol Chem (2005) 0.78
Emilin3 is required for notochord sheath integrity and interacts with Scube2 to regulate notochord-derived Hedgehog signals. Development (2013) 0.77
Characterization of a rare case of Ullrich congenital muscular dystrophy due to truncating mutations within the COL6A1 gene C-terminal domain: a case report. BMC Med Genet (2013) 0.77
Homer 2 antagonizes protein degradation in slow-twitch skeletal muscles. Am J Physiol Cell Physiol (2012) 0.75
Effects of oxygen concentration and pressure on Drosophila melanogaster: oxidative stress, mitochondrial activity, and survivorship. Arch Insect Biochem Physiol (2014) 0.75