Negamycin Restores Dystrophin Expression in Skeletal and Cardiac Muscles of mdx Mice
スポンサーリンク
概要
- 論文の詳細を見る
The ability of aminoglycoside antibiotics to promote read-through of nonsense muta diseases. However, the toxicity of aminoglycoside antibiotics may result in severe side effects during long-term treatment. In this paper, we report that negamycin, a dipeptide antibiotic, also restores dystrophin expression in skeletal and cardiac muscles of the mdx mouse, an animal model of Duchenne muscular dystrophy (DMD) with a nonsense mutation in the dystrophin gene, and in cultured mdx myotubes. Dystrophin expression was confirmed by immunohistochemistry and immunoblotting. We also compared the toxicity of negamycin and gentamicin, and found negamycin to be less toxic. Furthermore, we demonstrate that negamycin binds to a partial sequence of the eukaryotic rRNA-decoding A-site. We conclude that negamycin is a promising new therapeutic candidate for DMD and other genetic diseases caused by nonsense mutations.
- 社団法人 日本生化学会の論文
著者
-
HAMADA Masa
The Institute of Microbial Chemistry
-
IKEDA Daishiro
The Institute of Microbial Chemistry
-
TAKAHASHI Yoshikazu
The Institute of Microbial Chemistry
-
SAWA Ryuichi
The Institute of Microbial Chemistry
-
NONOMURA Yoshiaki
The Institute of Microbial Chemistry
-
Kaga Kimitaka
Department Of Otolaryngology School Of Medicine University Of Tokyo
-
Kitamura Toshio
Division Of Cellular Therapy Advanced Clinical Research Center Institute Of Medical Science Universi
-
Nakayama Yuki
Department Of Tumor Biochemistry Tokyo Metropolitan Institute Of Medical Sciences
-
Hara Takahiko
Department Of Computer Science And Intelligent Systems Oita University
-
Kondo Kenji
Department Of Electrical Engineering Faculty Of Science And Technology Keio University:fuji Electric
-
Arakawa Masayuki
Department Of Biochemistry And Cell Biology Institute Of Development And Aging Sciences Nippon Medic
-
Sheykholeslami Kianoush
Department Of Otolaryngology The University Of Tokyo
-
Shiozuka Masataka
Department Of Basic Human Sciences School Of Human Sciences Waseda University
-
Matsuda Ryoichi
Department of Applied Chemistry Faculty of Engineering Tokushima University
-
Suzuki-Miyagoe Yuko
Department of Molecular Therapy, National Institute of Neuroscience
関連論文
- ^As NMR Study of Hole-Doped Superconductor Ba_K_xFe_2As_2 (T_c≃38K)(Condensed matter: electronic structure and electrical, magnetic, and optical properties)
- Suppression of Magnetic Order by Pressure in BaFe_2As_2
- ^As NMR Study of the Ternary Iron Arsenide BaFe_2As_2(Condensed matter: electronic structure and electrical, magnetic, and optical properties)
- Efficient retroviral transduction of human B-lymphoid and myeloid progenitors : marked inhibition of their growth by the Pax5 transgene
- Immunohistochemical and genetic features of gastric and metastatic liver gastrointestinal stromal tumors : Sequential analyses
- Negamycin Restores Dystrophin Expression in Skeletal and Cardiac Muscles of mdx Mice
- A voice-generation system using an intramouth vibrator
- SPHINGOSINE-1-PHOSPHATE ACTIVATES QUIESCENT SATELLITE CELLS(Cell Biology and Morphology,Abstracts of papers presented at the 76^ Annual Meeting of the Zoological Society of Japan)
- Possible involvement of RasGRP4 in leukemogenesis
- Role of MgcRacGAP/Cyk4 as a Regulator of the Small GTPase Rho Family in Cytokinesis and Cell Differentiation
- Role of MgcRacGAP/Cyk4 as a Regulator of the small GTPase Rho Family in Cytokinesis and Cell Differentiation
- S-V-5 Satellite cell activation mediated with sphingosine-1-phosphate(The Proceedings of the 17th Annual Meetings of Japan Society of Exercise and Sports Physiology Jury 25-26, (Tokyo))
- MUSCLE SATELLITE CELL ACTIVATION IS ACCOMPANIED BY A DECREASE IN CELL SURFACE SPHINGOMYELIN(Developmental Biology,Abstracts of papers presented at the 74^ Annual Meeting of the Zoological Society of Japan)
- Synthesis of Negamycin and Its Derivatives as Potential Therapeutic Agents for Duchenne Muscular Dystrophy
- Glucose Homeostasis in a Diabetic Patient during Liver Transplantation : A Case Report
- An efficient method to prepare T cell receptor gene-transduced cytotoxic T lymphocytes type 1 applicable to tumor gene cell-therapy
- A Study of the Sustaining Mechanism in an Inductively Coupled Plasma ( Plasma Processing)
- Tacrolimus in corticosteroid-resistant ulcerative colitis
- GROWTH FACTOR ARRAY FABRICATION USING AN INK JET PRINTER II(Cell Biology and Morphology,Abstracts of papers presented at the 74^ Annual Meeting of the Zoological Society of Japan)
- Growth Factor Array Fabrication Using a Color Ink Jet Printer(Cell Biology)
- GROWTH FACTOR ARRAY FABRICATION USING AN INK JET PRINTER(Cell Biology and Morphology)(Proceedings of the Seventy-Third Annual Meeting of the Zoological Society of Japan)
- A method for gene transfer, single isolation and in vitro assay for neural stem cells
- OE-329 Novel Anti-Cell Death Protein (FNK) Therapy Preserves Myocardium Through Angiogenesis Mechanism After Myocardial Infarction in Swine(Regeneration (angionenesis/myocardial regeneration)-2 (M) OE55,Oral Presentation (English),The 70th Anniversary Ann
- Constitutive phosphorylation of a Rac GAP MgcRacGAP is implicated in v-Src-induced transformation of NIH3T3 cells
- Association of TP53 and MDM2 polymorphisms with survival in bladder cancer patients treated with chemoradiotherapy
- GROWTH FACTOR ARRAY FABRICATION USING AN INK JET PRINTER III(Developmental Biology,Abstracts of papers presented at the 75^ Annual Meeting of the Zoological Society of Japan)
- Cloning of a cDNA for Xenopus prolactin receptor and its metamorphic expression profile
- Activin A Inhibits Differentiation of Chick Myogenic Cells In Vitro
- DEVELOPMENT OF DUAL-REPORTER TRANSGENIC MICE FOR READTHROUGH ASSAY(Developmental Biology,Abstracts of papers presented at the 76^ Annual Meeting of the Zoological Society of Japan)
- ESTABLISHMENT OF MEASURING READTHROUGH ACTIVITY WITH MAMMALIAN CELL CULTURE(Biochemistry,Abstracts of papers presented at the 75^ Annual Meeting of the Zoological Society of Japan)
- SATELLITE CELL POOLS AND ACTIVATIONS IN THE MASSETER, GASTROCNEMIUS, SOLEUS, AND DIAPHRAGM MUSCLES OF MDX MICE(Developmental Biology,Abstracts of papers presented at the 75^ Annual Meeting of the Zoological Society of Japan)
- Self-Aligned Metal Double-Gate Low-Temperature Polycrystalline Silicon Thin-Film Transistors on Glass Substrate Using Back-Surface Exposure
- EFFECT OF TGF-β ON DIFFERENTIATION OF MYOGENIC SATELLITE CELLS(Developmental Biology,Abstracts of papers presented at the 74^ Annual Meeting of the Zoological Society of Japan)
- ROLES OF HGF/SF AND N-CADHERIN FOR MIGRATION OF SOMITIC MYOGENIC CELLS(Developmental Biology,Abstracts of papers presented at the 74^ Annual Meeting of the Zoological Society of Japan)
- ROLE OF HGF/C-MET SIGNALING IN SOMITE MYOGENESIS(Developmental Biology)(Proceedings of the Seventy-Third Annual Meeting of the Zoological Society of Japan)
- Diagnosis of primary versus metastatic ovarian adenocarcinoma using p53 gene mutation analysis
- Transdermal delivery of a readthrough-inducing drug : a new approach of gentamicin administration for the treatment of nonsense mutation-mediated disorders
- Negamycin can restore dystrophin in mdx mouse skeletal muscles in vivo and in vitro(Symposium on Current Research for Skeletal Muscle)
- Improved Serum-free Defined Medium for Proliferation and Differentiation of Chick Primary Myogenic Cells(Developmental Biology)
- Synthesis and Biological Evaluation of Dipeptidic Antibiotics (+)-Negamycin and Its Derivatives for Development of Duchenne Muscular Dystrophy Chemotherapy
- Aquaporin-4 is absent at the sarcolemma and at perivascular astrocyte endfeet in α1-syntrophin knockout mice
- Specific Protein Synthesis of sea Urchin Micromeres during Differentiation
- ECTOPIC CALCIFICATION IN MDX MOUSE SKELETAL MUSCLE (2)(Cell Biology and Morphology,Abstracts of papers presented at the 76^ Annual Meeting of the Zoological Society of Japan)
- Visualization of Dystrophic Muscle Fibers in Mdx Mouse by Vital Staining with Evans Blue: Evidence of Apoptosis in Dystrophin-Deficient Muscle
- Cloning of cDNA for newt WT1 and the differential expression during spermatogenesis of the Japanese newt, Cynops pyrrhogaster
- Cloning and Expression of a cDNA Encoding a Prolactin Receptor of the Japanese Red-Bellied Newt, Cynops pyrrhogaster(Endocrinology)
- Conversion from mitosis to meiosis : Morphology and expression of proliferating cell nuclear antigen (PCNA) and Dmc1 during newt spermatogenesis
- Newt RAD51 : Cloning of cDNA and analysis of gene expression during spermatogenesis
- Total hip arthroplasty with bone graft for acetabular protrusion in rheumatoid arthritis
- Unfused C2C12 Mouse Skeletal Muscle Cells Express Neurofilament 140K Protein
- IN VITRO MUSCLE FIBER FORMATION ON PATTERNED GELATIN STRIPES PRINTED BY A COLOR INK JET PRINTER(Cell Biology and Morphology,Abstracts of papers presented at the 75^ Annual Meeting of the Zoological Society of Japan)
- ELECTROMYOGRAPHIC EXAMINATION OF PATIENTS WITH UNILATERAL CORTICAL FACIAL PARALYSIS DUE TO LEFT FRONTAL LOBE INFARCTION : TWO CASES REPORT
- A NEW METHOD OF SPECIFIC INTRODUCTION OF SUBSTANCES INTO MYOTUBES USING MARCAINE(Cell Biology and Morphology,Abstracts of papers presented at the 75^ Annual Meeting of the Zoological Society of Japan)
- Fyn is not essential for Bcr-Abl-induced leukemogenesis in mouse bone marrow transplantation models
- Development of (+)-Negamycin Derivatives with Potent Readthrough Activity for Duchenne Muscular Dystrophy Chemotherapy
- Muscle injury-induced thymosin β4 acts as a chemoattractant for myoblasts
- Half-Quantum Vortices in Thin Film of Superfluid 3He
- Neuroendoscopic Transnasal Surgery for Skull Base Tumors : Basic Approaches, Avoidance of Pitfalls, and Recent Innovations
- Possible Multiple Gap Superconductivity with Line Nodes in Heavily Hole-Doped Superconductor KFe2As2 Studied by 75As Nuclear Quadrupole Resonance and Specific Heat
- MicroRNA-125b-1 accelerates a C-terminal mutant of C/EBPα (C/EBPα-C^m)-induced myeloid leukemia
- Kolbe's Reaction of Higher Fatty Acids. II. Electrolysis of Lauric Acid by a Pulsating Current with an Artificial Graphite Anode
- Upregulation of CD200R1 in lineage-negative leukemic cells is characteristic of AML1-ETO-positive leukemia in mice
- Negamycin Restores Dystrophin Expression in Skeletal and Cardiac Muscles of mdx Mice
- Structure Activity Relationship Study of Dipeptide Antibiotic (+)-Negamycin for the Potent Readthrough Promoting Activity