product summary
company name :
Leica Biosystems
other brands :
Novocastra, Surgipath
product type :
antibody
product name :
dystrophin
catalog :
NCL-DYS2
clonality :
monoclonal
host :
mouse
conjugate :
nonconjugated
clone name :
Dy8/6C5
reactivity :
human, mouse, rat, dogs
application :
western blot, immunohistochemistry, immunocytochemistry, immunohistochemistry - paraffin section, immunohistochemistry - frozen section, western blot knockout validation
more info or order :
citations: 46
Published Application/Species/Sample/DilutionReference
  • western blot knockout validation; mouse; 1:100; fig 1
Li D, Shin J, Duan D. iNOS ablation does not improve specific force of the extensor digitorum longus muscle in dystrophin-deficient mdx4cv mice. PLoS ONE. 2011;6:e21618 pubmed publisher
  • immunohistochemistry; dogs; 1:20; loading ...; fig 3
  • western blot; dogs; 1:40; loading ...; fig 2a
  • immunocytochemistry; human; loading ...; fig s20b
Amoasii L, Hildyard J, Li H, Sanchez Ortiz E, Mireault A, Caballero D, et al. Gene editing restores dystrophin expression in a canine model of Duchenne muscular dystrophy. Science. 2018;362:86-91 pubmed publisher
  • western blot; mouse; loading ...; fig 1a
Fukai Y, Ohsawa Y, Ohtsubo H, Nishimatsu S, Hagiwara H, Noda M, et al. Cleavage of ?-dystroglycan occurs in sarcoglycan-deficient skeletal muscle without MMP-2 and MMP-9. Biochem Biophys Res Commun. 2017;492:199-205 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; 1:100; fig 4c
Nelson C, Hakim C, Ousterout D, Thakore P, Moreb E, Castellanos Rivera R, et al. In vivo genome editing improves muscle function in a mouse model of Duchenne muscular dystrophy. Science. 2016;351:403-7 pubmed publisher
  • immunohistochemistry - paraffin section; human; 1:200; fig 4
Uruha A, Noguchi S, Hayashi Y, Tsuburaya R, Yonekawa T, Nonaka I, et al. Hepatitis C virus infection in inclusion body myositis: A case-control study. Neurology. 2016;86:211-7 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; 1:10; 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
  • immunocytochemistry; rat; fig 1
Rodríguez Muñoz R, Cárdenas Aguayo M, Alemán V, Osorio B, Chávez González O, Rendon A, et al. Novel Nuclear Protein Complexes of Dystrophin 71 Isoforms in Rat Cultured Hippocampal GABAergic and Glutamatergic Neurons. PLoS ONE. 2015;10:e0137328 pubmed publisher
  • western blot; human; 1:25
Ousterout D, Kabadi A, Thakore P, Perez Pinera P, Brown M, Majoros W, et al. Correction of dystrophin expression in cells from Duchenne muscular dystrophy patients through genomic excision of exon 51 by zinc finger nucleases. Mol Ther. 2015;23:523-32 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; mouse; 1:100; loading ...; fig 2,5
  • western blot; dogs; 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; mouse
Prins K, Lowe D, Ervasti J. Skeletal muscle-specific ablation of gamma(cyto)-actin does not exacerbate the mdx phenotype. PLoS ONE. 2008;3:e2419 pubmed publisher
You J, Dooley M, Kim C, Kim E, Xu W, Goodman C, et al. A DGKζ-FoxO-ubiquitin proteolytic axis controls fiber size during skeletal muscle remodeling. Sci Signal. 2018;11: 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
Huang L, Low A, Damle S, KEENAN M, Kuntz S, Murray S, et al. Antisense suppression of the nonsense mediated decay factor Upf3b as a potential treatment for diseases caused by nonsense mutations. Genome Biol. 2018;19:4 pubmed publisher
Randrianarison Huetz V, Papaefthymiou A, Herledan G, Noviello C, Faradova U, Collard L, et al. Srf controls satellite cell fusion through the maintenance of actin architecture. J Cell Biol. 2018;217:685-700 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
Sakakibara I, Wurmser M, Dos Santos M, Santolini M, Ducommun S, Davaze R, et al. Six1 homeoprotein drives myofiber type IIA specialization in soleus muscle. Skelet Muscle. 2016;6:30 pubmed publisher
Iyombe Engembe J, Ouellet D, Barbeau X, Rousseau J, Chapdelaine P, Lagüe P, et al. Efficient Restoration of the Dystrophin Gene Reading Frame and Protein Structure in DMD Myoblasts Using the CinDel Method. Mol Ther Nucleic Acids. 2016;5:e283 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
Palazzolo G, Quattrocelli M, Toelen J, Dominici R, Anastasia L, Tettamenti G, et al. Cardiac Niche Influences the Direct Reprogramming of Canine Fibroblasts into Cardiomyocyte-Like Cells. Stem Cells Int. 2016;2016:4969430 pubmed publisher
Murphy S, Henry M, Meleady P, Zweyer M, Mundegar R, Swandulla D, et al. Simultaneous Pathoproteomic Evaluation of the Dystrophin-Glycoprotein Complex and Secondary Changes in the mdx-4cv Mouse Model of Duchenne Muscular Dystrophy. Biology (Basel). 2015;4:397-423 pubmed publisher
Robriquet F, Lardenois A, Babarit C, Larcher T, Dubreil L, Leroux I, et al. Differential Gene Expression Profiling of Dystrophic Dog Muscle after MuStem Cell Transplantation. PLoS ONE. 2015;10:e0123336 pubmed publisher
Shimizu Motohashi Y, Asakura Y, Motohashi N, Belur N, Baumrucker M, Asakura A. Pregnancy-induced amelioration of muscular dystrophy phenotype in mdx mice via muscle membrane stabilization effect of glucocorticoid. PLoS ONE. 2015;10:e0120325 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
Larcher T, Lafoux A, Tesson L, Remy S, Thepenier V, François V, et al. Characterization of dystrophin deficient rats: a new model for Duchenne muscular dystrophy. PLoS ONE. 2014;9:e110371 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
Pakula A, Schneider J, Janke J, Zacharias U, Schulz H, Hubner N, et al. Altered expression of cyclin A 1 in muscle of patients with facioscapulohumeral muscle dystrophy (FSHD-1). PLoS ONE. 2013;8:e73573 pubmed publisher
Ousterout D, Perez Pinera P, Thakore P, Kabadi A, Brown M, Qin X, et al. Reading frame correction by targeted genome editing restores dystrophin expression in cells from Duchenne muscular dystrophy patients. Mol Ther. 2013;21:1718-26 pubmed publisher
Heydemann A, Swaggart K, Kim G, Holley Cuthrell J, Hadhazy M, McNally E. The superhealing MRL background improves muscular dystrophy. Skelet Muscle. 2012;2:26 pubmed publisher
Goyenvalle A, Babbs A, Wright J, Wilkins V, Powell D, Garcia L, et al. Rescue of severely affected dystrophin/utrophin-deficient mice through scAAV-U7snRNA-mediated exon skipping. Hum Mol Genet. 2012;21:2559-71 pubmed publisher
Arakawa R, Aoki R, Arakawa M, Saito K. Human first-trimester chorionic villi have a myogenic potential. Cell Tissue Res. 2012;348:189-97 pubmed publisher
Fragall C, Adams A, Johnsen R, Kole R, Fletcher S, Wilton S. Mismatched single stranded antisense oligonucleotides can induce efficient dystrophin splice switching. BMC Med Genet. 2011;12:141 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
Nishida A, Kataoka N, Takeshima Y, Yagi M, Awano H, Ota M, et al. Chemical treatment enhances skipping of a mutated exon in the dystrophin gene. Nat Commun. 2011;2:308 pubmed publisher
de Morrée A, Lutje Hulsik D, Impagliazzo A, van Haagen H, de Galan P, van Remoortere A, et al. Calpain 3 is a rapid-action, unidirectional proteolytic switch central to muscle remodeling. PLoS ONE. 2010;5:e11940 pubmed publisher
Rodino Klapac L, Montgomery C, Bremer W, Shontz K, Malik V, Davis N, et al. Persistent expression of FLAG-tagged micro dystrophin in nonhuman primates following intramuscular and vascular delivery. Mol Ther. 2010;18:109-17 pubmed publisher
Wu M, Katta A, Gadde M, Liu H, Kakarla S, Fannin J, et al. Aging-associated dysfunction of Akt/protein kinase B: S-nitrosylation and acetaminophen intervention. PLoS ONE. 2009;4:e6430 pubmed publisher
Friedrich O, von Wegner F, Chamberlain J, Fink R, Rohrbach P. L-type Ca2+ channel function is linked to dystrophin expression in mammalian muscle. PLoS ONE. 2008;3:e1762 pubmed publisher
Mattei E, Corbi N, Di Certo M, Strimpakos G, Severini C, Onori A, et al. Utrophin up-regulation by an artificial transcription factor in transgenic mice. PLoS ONE. 2007;2:e774 pubmed
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
Rodriguez A, Pisani D, Dechesne C, Turc Carel C, Kurzenne J, Wdziekonski B, et al. Transplantation of a multipotent cell population from human adipose tissue induces dystrophin expression in the immunocompetent mdx mouse. J Exp Med. 2005;201:1397-405 pubmed
Kogler G, Sensken S, Airey J, Trapp T, MUSCHEN M, Feldhahn N, et al. A new human somatic stem cell from placental cord blood with intrinsic pluripotent differentiation potential. J Exp Med. 2004;200:123-35 pubmed
Torrente Y, Tremblay J, Pisati F, Belicchi M, Rossi B, Sironi M, et al. Intraarterial injection of muscle-derived CD34(+)Sca-1(+) stem cells restores dystrophin in mdx mice. J Cell Biol. 2001;152:335-48 pubmed
product information
product code :
NCL-DYS2
product name :
dystrophin
application :
IHCF
more info or order :
company information
Leica Biosystems
hrna@leica-microsystems.com
http://www.leicabiosystems.com