| Published Application/Species/Sample/Dilution | Reference |
|---|
- western blot; pigs ; 1:100; loading ...; fig 2d
| Echigoya Y, Trieu N, Duddy W, Moulton H, Yin H, Partridge T, et al. A Dystrophin Exon-52 Deleted Miniature Pig Model of Duchenne Muscular Dystrophy and Evaluation of Exon Skipping. Int J Mol Sci. 2021;22: pubmed publisher |
- western blot; mouse; 1:1500; loading ...; fig 3d
| Li D, Adams A, Johnsen R, Fletcher S, Wilton S. Morpholino Oligomer-Induced Dystrophin Isoforms to Map the Functional Domains in the Dystrophin Protein. Mol Ther Nucleic Acids. 2020;22:263-272 pubmed publisher |
- immunocytochemistry; human; 1:500; loading ...; fig 10a
| Fulgenzi G, Hong Z, Tomassoni Ardori F, Barella L, Becker J, Barrick C, et al. Novel metabolic role for BDNF in pancreatic β-cell insulin secretion. Nat Commun. 2020;11:1950 pubmed publisher |
- immunohistochemistry - frozen section; human; loading ...
- western blot; human; loading ...
| Papadopoulos C, Laforet P, Nectoux J, Stojkovic T, Wahbi K, Carlier R, et al. Hyperckemia and myalgia are common presentations of anoctamin-5-related myopathy in French patients. Muscle Nerve. 2017;56:1096-1100 pubmed publisher |
- western blot; mouse; 1:1000; loading ...; fig 1d
| Vila M, Rayavarapu S, Hogarth M, van der Meulen J, Horn A, Defour A, et al. Mitochondria mediate cell membrane repair and contribute to Duchenne muscular dystrophy. Cell Death Differ. 2017;24:330-342 pubmed publisher |
- western blot; human; 1:40; fig 1
| Martone J, Briganti F, Legnini I, Morlando M, Picillo E, Sthandier O, et al. The lack of the Celf2a splicing factor converts a Duchenne genotype into a Becker phenotype. Nat Commun. 2016;7:10488 pubmed publisher |
- western blot; mouse; 1:100; loading ...; fig 3b
| Tabebordbar M, Zhu K, Cheng J, Chew W, Widrick J, Yan W, et al. In vivo gene editing in dystrophic mouse muscle and muscle stem cells. Science. 2016;351:407-411 pubmed publisher |
- immunocytochemistry; dogs; fig 6d
| Loperfido M, Jarmin S, Dastidar S, Di Matteo M, Perini I, Moore M, et al. piggyBac transposons expressing full-length human dystrophin enable genetic correction of dystrophic mesoangioblasts. Nucleic Acids Res. 2016;44:744-60 pubmed publisher |
- immunohistochemistry; human; 1:5; loading ...; tbl 1
| Shah F, Berggren D, Holmlund T, Levring Jäghagen E, Stål P. Unique expression of cytoskeletal proteins in human soft palate muscles. J Anat. 2016;228:487-94 pubmed publisher |
- immunohistochemistry - frozen section; mouse; 1:50; fig 3
| He Q, Duguid I, Clark B, Panzanelli P, Patel B, Thomas P, et al. Interneuron- and GABA(A) receptor-specific inhibitory synaptic plasticity in cerebellar Purkinje cells. Nat Commun. 2015;6:7364 pubmed publisher |
- immunohistochemistry - frozen section; human; 1:50
| Screen M, Jonson P, Raheem O, Palmio J, Laaksonen R, Lehtimaki T, et al. Abnormal splicing of NEDD4 in myotonic dystrophy type 2: possible link to statin adverse reactions. Am J Pathol. 2014;184:2322-32 pubmed publisher |
- western blot; human; loading ...; fig 3a
| Wang Y, Mariño Enríquez A, Bennett R, Zhu M, Shen Y, Eilers G, et al. Dystrophin is a tumor suppressor in human cancers with myogenic programs. Nat Genet. 2014;46:601-6 pubmed publisher |
- western blot; dogs; 1:100; loading ...; fig 2,5
- western blot; mouse; 1:100; loading ...; fig 2,5
| Kodippili K, Vince L, Shin J, Yue Y, Morris G, McIntosh M, et al. Characterization of 65 epitope-specific dystrophin monoclonal antibodies in canine and murine models of duchenne muscular dystrophy by immunostaining and western blot. PLoS ONE. 2014;9:e88280 pubmed publisher |
- western blot; rhesus macaque; loading ...; fig 7
| Chicoine L, Rodino Klapac L, Shao G, Xu R, Bremer W, Camboni M, et al. Vascular delivery of rAAVrh74.MCK.GALGT2 to the gastrocnemius muscle of the rhesus macaque stimulates the expression of dystrophin and laminin ?2 surrogates. Mol Ther. 2014;22:713-24 pubmed publisher |
| Lorant J, Larcher T, Jaulin N, Hedan B, Lardenois A, Leroux I, et al. Vascular Delivery of Allogeneic MuStem Cells in Dystrophic Dogs Requires Only Short-Term Immunosuppression to Avoid Host Immunity and Generate Clinical/Tissue Benefits. Cell Transplant. 2018;27:1096-1110 pubmed publisher |
| Mata López S, Hammond J, Rigsby M, Balog Alvarez C, Kornegay J, Nghiem P. A novel canine model for Duchenne muscular dystrophy (DMD): single nucleotide deletion in DMD gene exon 20. Skelet Muscle. 2018;8:16 pubmed publisher |
| Sánchez L, Beltran E, De Stefani A, Guo L, Shea A, Shelton G, et al. Clinical and genetic characterisation of dystrophin-deficient muscular dystrophy in a family of Miniature Poodle dogs. PLoS ONE. 2018;13:e0193372 pubmed publisher |
| Carlson C, Moore S, Mathews K. Dystrophinopathy muscle biopsies in the genetic testing ERA: One center's data. Muscle Nerve. 2018;: pubmed publisher |
| Echigoya Y, Nakamura A, Nagata T, Urasawa N, Lim K, Trieu N, et al. Effects of systemic multiexon skipping with peptide-conjugated morpholinos in the heart of a dog model of Duchenne muscular dystrophy. Proc Natl Acad Sci U S A. 2017;114:4213-4218 pubmed publisher |
| Suriyonplengsaeng C, Dejthevaporn C, Khongkhatithum C, Sanpapant S, Tubthong N, Pinpradap K, et al. Immunohistochemistry of sarcolemmal membrane-associated proteins in formalin-fixed and paraffin-embedded skeletal muscle tissue: a promising tool for the diagnostic evaluation of common muscular dystrophies. Diagn Pathol. 2017;12:19 pubmed publisher |
| Roy P, Rau F, Ochala J, Messéant J, Fraysse B, Laine J, et al. Dystrophin restoration therapy improves both the reduced excitability and the force drop induced by lengthening contractions in dystrophic mdx skeletal muscle. Skelet Muscle. 2016;6:23 pubmed publisher |
| Toh Z, Thandar Aung Htut M, Pinniger G, Adams A, Krishnaswarmy S, Wong B, et al. Deletion of Dystrophin In-Frame Exon 5 Leads to a Severe Phenotype: Guidance for Exon Skipping Strategies. PLoS ONE. 2016;11:e0145620 pubmed publisher |
| Massouridès E, Polentes J, Mangeot P, Mournetas V, Nectoux J, Deburgrave N, et al. Dp412e: a novel human embryonic dystrophin isoform induced by BMP4 in early differentiated cells. Skelet Muscle. 2015;5:40 pubmed publisher |
| Steffen F, Bilzer T, Brands J, Golini L, Jagannathan V, Wiedmer M, et al. A Nonsense Variant in COL6A1 in Landseer Dogs with Muscular Dystrophy. G3 (Bethesda). 2015;5:2611-7 pubmed publisher |
| Rouillon J, Poupiot J, Zocevic A, Amor F, Léger T, García C, et al. Serum proteomic profiling reveals fragments of MYOM3 as potential biomarkers for monitoring the outcome of therapeutic interventions in muscular dystrophies. Hum Mol Genet. 2015;24:4916-32 pubmed publisher |
| Li X, Zhao L, Zhou S, Hu C, Shi Y, Shi W, et al. A comprehensive database of Duchenne and Becker muscular dystrophy patients (0-18 years old) in East China. Orphanet J Rare Dis. 2015;10:5 pubmed publisher |
| Gandolfi B, Daniel R, O Brien D, Guo L, Youngs M, Leach S, et al. A novel mutation in CLCN1 associated with feline myotonia congenita. PLoS ONE. 2014;9:e109926 pubmed publisher |
| Cotta A, Paim J, da Cunha Junior A, Neto R, Nunes S, Navarro M, et al. Limb girdle muscular dystrophy type 2G with myopathic-neurogenic motor unit potentials and a novel muscle image pattern. BMC Clin Pathol. 2014;14:41 pubmed publisher |
| Mahmood O, Jiang X, Zhang Q. Limb-girdle muscular dystrophy subtypes: First-reported cohort from northeastern China. Neural Regen Res. 2013;8:1907-18 pubmed publisher |
| Verhaart I, van Vliet van den Dool L, Sipkens J, De Kimpe S, Kolfschoten I, van Deutekom J, et al. The Dynamics of Compound, Transcript, and Protein Effects After Treatment With 2OMePS Antisense Oligonucleotides in mdx Mice. Mol Ther Nucleic Acids. 2014;3:e148 pubmed publisher |
| Johnson E, Li B, Yoon J, Flanigan K, Martin P, ERVASTI J, et al. Identification of new dystroglycan complexes in skeletal muscle. PLoS ONE. 2013;8:e73224 pubmed publisher |
| Roberts T, Godfrey C, McClorey G, Vader P, Briggs D, Gardiner C, et al. Extracellular microRNAs are dynamic non-vesicular biomarkers of muscle turnover. Nucleic Acids Res. 2013;41:9500-13 pubmed publisher |
| Raith M, Valencia R, Fischer I, Orthofer M, Penninger J, Spuler S, et al. Linking cytoarchitecture to metabolism: sarcolemma-associated plectin affects glucose uptake by destabilizing microtubule networks in mdx myofibers. Skelet Muscle. 2013;3:14 pubmed publisher |
| Tanaka A, Woltjen K, Miyake K, Hotta A, Ikeya M, Yamamoto T, et al. Efficient and reproducible myogenic differentiation from human iPS cells: prospects for modeling Miyoshi Myopathy in vitro. PLoS ONE. 2013;8:e61540 pubmed publisher |
| Araujo K, Bonuccelli G, Duarte C, Gaiad T, Moreira D, Feder D, et al. Bortezomib (PS-341) treatment decreases inflammation and partially rescues the expression of the dystrophin-glycoprotein complex in GRMD dogs. PLoS ONE. 2013;8:e61367 pubmed publisher |
| Fletcher S, Adkin C, Meloni P, Wong B, Muntoni F, Kole R, et al. Targeted exon skipping to address "leaky" mutations in the dystrophin gene. Mol Ther Nucleic Acids. 2012;1:e48 pubmed publisher |
| Tanganyika de Winter C, Heemskerk H, Karnaoukh T, van Putten M, De Kimpe S, van Deutekom J, et al. Long-term Exon Skipping Studies With 2'-O-Methyl Phosphorothioate Antisense Oligonucleotides in Dystrophic Mouse Models. Mol Ther Nucleic Acids. 2012;1:e44 pubmed publisher |
| van Putten M, Hulsker M, Nadarajah V, van Heiningen S, van Huizen E, van Iterson M, et al. The effects of low levels of dystrophin on mouse muscle function and pathology. PLoS ONE. 2012;7:e31937 pubmed publisher |
| Graciotti L, Becker J, Granata A, Procopio A, Tessarollo L, Fulgenzi G. Dystrophin is required for the normal function of the cardio-protective K(ATP) channel in cardiomyocytes. PLoS ONE. 2011;6:e27034 pubmed publisher |
| Bortolanza S, Nonis A, Sanvito F, Maciotta S, Sitia G, Wei J, et al. AAV6-mediated systemic shRNA delivery reverses disease in a mouse model of facioscapulohumeral muscular dystrophy. Mol Ther. 2011;19:2055-64 pubmed publisher |
| Saito T, Nakamura A, Aoki Y, Yokota T, Okada T, Osawa M, et al. Antisense PMO found in dystrophic dog model was effective in cells from exon 7-deleted DMD patient. PLoS ONE. 2010;5:e12239 pubmed publisher |
| Chandrasekharan K, Yoon J, Xu Y, DeVries S, Camboni M, Janssen P, et al. A human-specific deletion in mouse Cmah increases disease severity in the mdx model of Duchenne muscular dystrophy. Sci Transl Med. 2010;2:42ra54 pubmed publisher |
| Heemskerk H, de Winter C, van Kuik P, Heuvelmans N, Sabatelli P, Rimessi P, et al. Preclinical PK and PD studies on 2'-O-methyl-phosphorothioate RNA antisense oligonucleotides in the mdx mouse model. Mol Ther. 2010;18:1210-7 pubmed publisher |
| Van Vliet L, De Winter C, van Deutekom J, van Ommen G, Aartsma Rus A. Assessment of the feasibility of exon 45-55 multiexon skipping for Duchenne muscular dystrophy. BMC Med Genet. 2008;9:105 pubmed publisher |
| Aartsma Rus A, Janson A, van Ommen G, van Deutekom J. Antisense-induced exon skipping for duplications in Duchenne muscular dystrophy. BMC Med Genet. 2007;8:43 pubmed |
| Iwata Y, Katanosaka Y, Arai Y, Komamura K, Miyatake K, Shigekawa M. A novel mechanism of myocyte degeneration involving the Ca2+-permeable growth factor-regulated channel. J Cell Biol. 2003;161:957-67 pubmed |
