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DystrophinAntibodiesMuscular Dystrophy, DuchenneUtrophinMice, Inbred mdxMuscular Dystrophy, AnimalAntibody SpecificityMuscular DystrophiesDystrophin-Associated ProteinsAntibodies, ViralAntibodies, MonoclonalAntibodies, BacterialAntibody FormationAntibodies, NeutralizingDystroglycansFluorescent Antibody TechniqueSarcolemmaAntibody AffinityAntibodies, Anti-IdiotypicBinding Sites, AntibodyMolecular Sequence DataSarcoglycansMuscle, SkeletalEpitopesHIV AntibodiesAntibodies, NeoplasmAntibodies, ProtozoanAmino Acid SequenceAntibodies, AntinuclearExonsCross ReactionsCytoskeletal ProteinsMusclesMuscle ProteinsDystrophin-Associated Protein ComplexMorpholinosImmunoglobulin MAutoantibodiesImmunohistochemistryAntibodies, FungalMuscle Fibers, SkeletalBlotting, WesternNeutralization TestsMice, Inbred C57BLRabbitsBase SequenceAntigen-Antibody ReactionsAntibodies, BispecificMembrane ProteinsMice, Inbred BALB COligoribonucleotides, AntisenseSingle-Chain AntibodiesImmunoblottingAntibodies, BlockingImmunoglobulin GAntigen-Antibody ComplexImmunoglobulin Fab FragmentsCell LineFluorescent Antibody Technique, IndirectAntibodies, HeterophileAntibodies, CatalyticCells, CulturedSpectrinGenetic TherapyEpitope MappingElectrophoresis, Polyacrylamide GelImmune SeraImmunoglobulin AMolecular WeightMembrane GlycoproteinsAntibodies, Monoclonal, HumanizedHybridomasRecombinant ProteinsImmunoenzyme TechniquesCaveolin 3ActininAntibodies, AntiphospholipidDisease Models, AnimalImmunizationProtein BindingRecombinant Fusion ProteinsEnzyme-Linked Immunosorbent AssayCardiomyopathy, DilatedAntigensPolymerase Chain ReactionReading FramesMutationAntigens, BacterialSequence DeletionGlycoproteinsAntigens, SurfaceRNA, MessengerOligonucleotides, AntisenseImmunization, PassivePhenotypeGenetic VectorsImmunoassayImmunoglobulin FragmentsTime FactorsPeptide Fragments
Component of the dystrophin-glycoprotein complexGenePolyclonal AntibodyGlycoproteinMicro-dystrophin proteinDuchenneDouble AntibodyMonoclonal AntibodiesSkeletalExtracellular matrixSandwich ELISAProteinsRecombinantMuscleMiceSpecific antibodiesPeptideWestern blotBiotinMuscularFITCImmunofluorescenceRabbitAlpha dystroglycanClinicalDetectionReactsSpeciesAssaysDystroglycanSerumAlexa FluorDesminProtein presentEpitopeDilutionLamininStainMembraneProducts
Component of the dystrophin-glycoprotein complex2
  • Dystroglycan is a laminin binding component of the dystrophin-glycoprotein complex which provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. (thermofisher.com)
  • We have partially sequenced rabbit skeletal muscle γ-sarcoglycan an integral component of the dystrophin-glycoprotein complex. (elsevierpure.com)
Gene22
  • Product Name/ID: MANEX1216D(8D11) Available to For-Profits: Yes Alternate Antibody Name: Gene Name: DMD Ab Isotype: MIgG2a Gene Symbol: Antibo. (uiowa.edu)
  • In vivo targeted repair of a point mutation in the canine dystrophin gene by a chimeric RNA/DNA oligonucleotide. (uiowa.edu)
  • The large size of the gene makes it more vulnerable to mutations resulting in partially functional or non-functional Dystrophin. (iit.edu)
  • The disease occurs due to a defective gene that results in absence of dystrophin, a protein that helps keep the body's muscle cells intact. (scienceboard.net)
  • Elevidys is a recombinant gene therapy designed to deliver a gene into the body that leads to production of Elevidys micro-dystrophin, a shortened protein (138 kDa, compared to the 427 kDa dystrophin protein of normal muscle cells) that contains selected domains of the dystrophin protein present in normal muscle cells. (scienceboard.net)
  • It is caused by mutations in the gene encoding the protein dystrophin. (delveinsight.com)
  • Gene replacement or other genetic therapies linked to specific mutations to restore dystrophin production, membrane stabilization and/or upregulation of compensatory proteins, and reduction of the inflammatory cascade and/or enhancement of muscle regeneration are the main therapeutic strategies for DMD treatment . (delveinsight.com)
  • ELEVIDYS addresses the core genetic cause of Duchenne, mutations in the dystrophin gene that result in a lack of dystrophin protein, by delivering to muscle cells an ELEVIDYS micro-dystrophin gene that codes for a shorter form of dystrophin. (delveinsight.com)
  • Duchenne muscular dystrophy (DMD), the most common and severe form of muscular dystrophy, is caused by mutations in the dystrophin gene, the largest gene identified in the human genome. (biomedcentral.com)
  • The goal for this new gene is to give the cells instructions in hopes of making a working version of the dystrophin protein. (duchenne.com)
  • The dystrophin gene, or DMD gene, is the largest in the human body, which can make it difficult to deliver to cells. (duchenne.com)
  • This allows them to see if the combination they chose for the new gene can help cells produce dystrophin. (duchenne.com)
  • This is accomplished through a genetic test that looks for a change, or mutation, in the dystrophin gene. (duchenne.com)
  • This test measures whether you have a type of antibody that could block gene therapy from working as intended, which could also impact safety. (duchenne.com)
  • If someone has been previously exposed to a virus similar to a gene therapy's vector, the immune system's antibodies may prevent the gene therapy from doing its job and could cause harmful side effects. (duchenne.com)
  • Remember, genetic and preexisting antibody testing are only 2 elements that may be required to determine eligibility for gene therapy-your doctor can provide more information about additional requirements for specific treatments. (duchenne.com)
  • Read more about antibody testing and gene therapy. (duchenne.com)
  • Researchers are looking at whether or not gene therapy can help the body make a different but working version of dystrophin. (duchenne.com)
  • CRISPR-Cas9, a gene-editing tool discovered at Berkeley in 2014, has been shaking up therapeutics from cancer to heart disease and has offered the promise of targeting the mutated dystrophin gene itself. (berkeley.edu)
  • We have recently cloned the gene for the murine 87 kDa postsynaptic protein dystrobrevin, a dystrophin-associated protein. (ox.ac.uk)
  • DMD is caused by mutation of the dystrophin gene ( DMD ), which resides on the X chromosome 1 , 2 . (plos.org)
  • It was during his time as a post-doctoral fellow, eight years after the discovery of dystrophin as the etiology of DMD, that alpha sarcoglycan was discovered in Dr. Campbell's lab as the second gene for a muscle disease. (worldmusclesociety.org)
Polyclonal Antibody3
  • Description: A polyclonal antibody for detection of Met from Human, Mouse, Rat. (applesci.com)
  • The following product was used in this experiment: Dystroglycan Polyclonal Antibody from Thermo Fisher Scientific, catalog # 11017-1-AP. (thermofisher.com)
  • The polyclonal antibody you generated for KIAA0408 is stunning! (bicellscientific.com)
Glycoprotein4
  • It plays a vital role in localizing the Dystrophin glycoprotein complex (DGC) in the Sarcollema and is associated with the DGC in controlling the signaling events of certain proteins associated with DGC. (iit.edu)
  • Component of the dystrophin- associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. (assaygenie.com)
  • This period of Professor Straub's career, given his deep involvement in this early research, where diseases localising to the dystrophin-glycoprotein complex, especially limb-girdle muscular dystrophies, began to hold a special place in his heart as his favorite group of neuromuscular diseases. (worldmusclesociety.org)
  • They are associated with altered glycosylation of the dystrophin-glycoprotein (DGC) complex components, such as alpha-dystroglycan (alpha-DG), fundamental in the structural and functional stability of the muscle fiber. (cnr.it)
Micro-dystrophin protein2
  • Data obtained from a randomized clinical trial established that Elevidys increased the expression of the Elevidys micro-dystrophin protein observed in Elevidys-treated individuals 4 to 5 years of age with DMD. (scienceboard.net)
  • This fast approval is predicated on an increase in skeletal muscle ELEVIDYS micro-dystrophin protein expression. (delveinsight.com)
Duchenne7
  • DMD (Dystrophin) therapy is the only FDA-approved treatment for Duchenne muscular dystrophy (DMD), a rare, progressive muscle disorder that affects boys and young men. (assaygenie.com)
  • Dystrophin/utrophin double-knockout (dKO) mice develop a extra extreme and progressive muscular dystrophy than the mdx mice, the commonest murine mannequin of Duchenne muscular dystrophy (DMD). (aprofarm.org)
  • With Duchenne, the body doesn't make enough dystrophin, a protein muscles need to work properly. (duchenne.com)
  • Duchenne is caused by an absence of dystrophin, a huge protein on the X chromosome that helps the body form healthy muscle tissue. (berkeley.edu)
  • Mutations in the genes encoding dystrophin or dystrophin-associated proteins are responsible for Duchenne muscular dystrophy or various forms of limb-girdle muscular dystrophies respectively. (ox.ac.uk)
  • By contrast, dystrobrevin membrane staining is severely reduced in muscles of Duchenne muscular dystrophy patients, consistent with dystrobrevin being a dystrophin-associated protein. (ox.ac.uk)
  • The protein made from the human Duchenne muscular dystrophy locus (DMD) and its mouse homolog (mDMD) had been identified with the aid of the use of polyclonal antibodies directed in opposition to fusion proteins containing two wonderful areas of the mDMD cDNA. (technewz.co.in)
Double Antibody2
Monoclonal Antibodies4
  • The diagnosis of dystroglycanopathies is currently based on the observation of clinical manifestations, muscle biopsies and enzymatic measures, and the available monoclonal antibodies are not specific for the dystrophic hypoglycosylated muscle condition. (cnr.it)
  • Glycopeptide 1 was used for the development of polyclonal antibodies and recombinant monoclonal antibodies by Phage Display technology. (cnr.it)
  • Regarding monoclonal antibodies, for the first time variable heavy (VH) and variable light (VL) immunoglobulin domains were selected by Phage Display, identified by NGS and described by in silico analysis. (cnr.it)
  • Production and characterization of monoclonal antibodies to the extracellular domain of P0. (2medicalcare.com)
Skeletal3
  • Treatment with the anti-IL-6 receptor antibody attenuates muscular dystrophy via promoting skeletal muscle regeneration in dystrophin-/utrophin-deficient mice. (aprofarm.org)
  • Specific antibodies were produced against a γ-sarcoglycan peptide and used to examine the expression of γ-sarcoglycan in skeletal muscle of patients with severe childhood autosomal muscular dystrophy linked to chromosome 13q12 (SCARMD). (elsevierpure.com)
  • Anti-dystrobrevin antibodies stain the sarcolemma in normal skeletal muscle indicating that dystrobrevin co-localises with dystrophin and the dystrophin-associated protein complex. (ox.ac.uk)
Extracellular matrix3
  • The absence of Dystrophin results in disruption of sub sarcolemma-extracellular matrix linkage, loss of nitric oxide, progressive muscle weakening and muscle wasting leading to the death of patients typically before the end of their teenage. (iit.edu)
  • dystrophin: Anchors the extracellular matrix to the cytoskeleton via F-actin. (assaygenie.com)
  • Muscle fibers formed by donated cells were labeled with human-specific antibody dystrophin, and the secreted extracellular matrix was labeled with laminin. (visikol.com)
Sandwich ELISA1
  • Description: A sandwich ELISA kit for detection of Dystrophin from Human in samples from blood, serum, plasma, cell culture fluid and other biological fluids. (tissue-cell-culture.com)
Proteins4
  • Dystrophin is an important cytoskeletal protein which belongs to the β-Spectrin/α-actinin family of proteins. (iit.edu)
  • Syntrophins are cytoplasmic peripheral membrane proteins that bind to components of mechanosenstive sodium channels and the extreme carboxy-terminal domain of dystrophin and dystrophin-related proteins. (antibodies-online.com)
  • Gamma1- and gamma2-syntrophins, two novel dystrophin-binding proteins localized in neuronal cells. (antibodies-online.com)
  • Our results show that dystrobrevin-deficiency is a generic feature of dystrophies linked to dystrophin and the dystrophin-associated proteins. (ox.ac.uk)
Recombinant3
  • Recombinant monoclonal antibody to Beta Dystroglycan. (absoluteantibody.com)
  • This antibody can also be used for the identification human beta-DG, mouse beta-DG and recombinant beta dystroglycan in a western blot. (absoluteantibody.com)
  • Evinacumab is a recombinant human monoclonal antibody that binds to and inhibits angiopoietin-like 3 (ANGPTL3). (medscape.com)
Muscle7
  • Restoration of pharyngeal dilator muscle force in dystrophin-deficient (mdx) mice following co-treatment with neutralizing interleukin-6 receptor antibodies and urocortin 2. (nih.gov)
  • KRT8 together with KRT19, help to link the contractile apparatus to dystrophin at the costameres of striated muscle. (avivasysbio.com)
  • This antibody was also used for the immunofluorescent staining of unfixed human and mouse muscle tissues. (absoluteantibody.com)
  • Intramuscular injection of donor muscle-derived cells into chimeric cxmd recipients restored dystrophin expression for at least 24 weeks in the absence of post-transplant immunosuppression, indicating that cell transplantation may be a viable therapeutic option for muscular dystrophy. (biomedcentral.com)
  • Diprotin A stimulation of CXCR4, however, significantly increased the number of canine dystrophin-positive muscle fibers and canine-derived satellite cells by enhancing donor cell proliferation. (biomedcentral.com)
  • Syncoilin links the dystrophin associated protein complex (DAPC) to desmin filaments in muscle and plays a structural role in striated muscle. (bicellscientific.com)
  • The dystrophin-deficiency leads to severe muscle lesions including necrosis, regeneration, inflammation, fibrosis and fatty degeneration. (researchsquare.com)
Mice2
  • As an alternative, nonhuman AdV (NHAdV) vectors can circumvent neutralizing antibodies against HAdVs in immunized mice and monkeys and in human sera, suggesting that NHAdV vectors could circumvent preexisting humoral immunity against HAdVs in a clinical setting. (irb.hr)
  • The original antibody was generated by immunizing Balb/c and Trianni mice with human beta-dystroglycan. (absoluteantibody.com)
Specific antibodies4
  • By applying specific antibodies and using double-labeling techniques, we have identified macrophages, degenerating myofibers, fibroblasts, split myofibers and new myobalsts. (cdc.gov)
  • When someone has been exposed to a virus, the body's immune system creates specific antibodies that remember how to fight it in the future. (duchenne.com)
  • Scientists are in the early stages of research to address this, and are working hard to see if there's a way around the specific antibodies that recognize the vector. (duchenne.com)
  • Human-specific antibodies were used for marking so recovered cells could then be tested using a PCR for their human-specific markers to prove they were not of mouse origin. (visikol.com)
Peptide1
Western blot4
  • Anti-Syncoilin (SYNC) antibody is validated on mouse tissue and recommended for immunofluorescence labeling, IHC, or western blot of materials from rodent and human tissues. (bicellscientific.com)
  • Western blot-specific recognition antibodies are made against the same epitope sequence with novel denaturing and stabilizing technique to prevent the natural folding of epitope in order to lock its conformation in unfolded state. (bicellscientific.com)
  • Absence of dystrophin, dysferlin, or α-sarcoglycan protein was confirmed by western blot and immunohistochemistry. (plos.org)
  • Western blot: 10 µg of MCF7, Caco-2, HaCat and PC-3 cell lysates stained with ARG66324 anti-Cytokeratin 19 antibody [SQab1872] at 1:1000 dilution. (arigobio.cn)
Biotin1
  • CusabioALB Antibody, Biotin conjugated is Available at Gentaur Genprice with the fastest delivery.Online Order Payment is possible or send. (joplink.net)
Muscular3
  • La Miopatía de Bethlem (MB) y la Distrofia Muscular Congénita de Ullrich (DMCU) son resultados de una mutación en el colágeno VI. (bvsalud.org)
  • Normal dystrobrevin staining is observed in patients with other forms of limb-girdle muscular dystrophy where dystrophin and the rest of the dystrophin-associated protein complex are normally expressed and in other neuromuscular disorders. (ox.ac.uk)
  • This is the first indication that a cytoplasmic component of the dystrophin-associated protein complex may be involved in the pathogenesis of limb-girdle muscular dystrophy. (ox.ac.uk)
FITC1
  • CusabioALB Antibody, FITC conjugated is Available at Gentaur Genprice with the fastest delivery.Online Order Payment is possible or send quotation to. (joplink.net)
Immunofluorescence1
  • This antibody does not work for Immunofluorescence. (uiowa.edu)
Rabbit2
  • In general, rabbit antibodies demonstrate greater affinity and are used at a magnitude lower Ig concentration for initial testing. (uiowa.edu)
  • This Met antibody is for IHC-P. It is affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogenand is unconjugated. (applesci.com)
Alpha dystroglycan3
  • The binding of this antibody to beta dystroglycan does not affect its binding to alpha dystroglycan. (absoluteantibody.com)
  • It plays an important role in linking dystrophin to the laminin-binding alpha-dystroglycan. (absoluteantibody.com)
  • This antibody is capable of immunoprecipitating both alpha-DG and beta-DG from C2C12 cell lysates, suggesting that the binding epitope of this antibody on beta dystroglycan is different from that of alpha dystroglycan. (absoluteantibody.com)
Clinical1
  • these are individuals who do not have significant preexisting antibody titers against the AAV rh74 vector or have other contraindications based on the inclusion criteria of the clinical trials. (scienceboard.net)
Detection2
  • Description: A sandwich quantitative ELISA assay kit for detection of Human Dystrophin (DMD) in samples from tissue homogenates, cell lysates or other biological fluids. (tissue-cell-culture.com)
  • Detection and quantification of antibodies to the extracellular domain of P0 during experimental allergic neuritis. (2medicalcare.com)
Reacts1
Species1
Assays1
  • Accordingly, polyclonal antibodies were reactive to glycopeptide 1, which enables the application of anti-glycopeptide 1 antibodies in immune reactive assays targeting hypoglycosylated alpha-DG. (cnr.it)
Dystroglycan2
  • This antibody recognizes human and mouse beta dystroglycan (β-DAG). (absoluteantibody.com)
  • The binding specificity of this antibody for human and mouse beta dystroglycan (beta-DG) was confirmed using ELISA. (absoluteantibody.com)
Serum1
  • The Assay Genie Human Dystrophin ELISA is a highly sensitive assay for the quantitative measurement of Dystrophin in serum, blood, plasma, cell culture supernatant and tissue samples. (assaygenie.com)
Alexa Fluor1
  • The cells were stained with ARG66324 anti-Cytokeratin 19 antibody [SQab1872] (red) at 1:200 dilution in 1x PBS/1% BSA for 30 min at RT, followed by Alexa Fluor® 488 labelled secondary antibody. (arigobio.cn)
Desmin1
  • Myoblasts can be visualized using desmin and dystrophin or desmin and vimentin. (cdc.gov)
Protein present1
Epitope1
  • FFPE-specific recognition antibodies are made against the same epitope sequence with novel cross-linking and stabilizing technique to lock the conformational state of epitope in a folded state similar to aldehyde induced fixation. (bicellscientific.com)
Dilution1
  • Immunohistochemistry: Formalin-fixed and paraffin-embedded thyroid cancer tissue stained with ARG66324 anti-Cytokeratin 19 antibody [SQab1872] at 1:40,000 dilution. (arigobio.cn)
Laminin1
  • This antibody was used in combination with anti-laminin-2 antibody C3_Hu10 to generate a duobody called TBTI (US11254737). (absoluteantibody.com)
Stain1
  • Cells were stained with ARG66324 anti-Cytokeratin 19 antibody [SQab1872] (green) at 1:40,000 and 4°C. DAPI (blue) was used as the nuclear counter stain. (arigobio.cn)
Membrane1
  • Professor Straub's PhD work was aimed at identifying the sub-cellular organisation of dystrophin using these antibodies and involved much work with complicated electron microscopy techniques (DOI: 10.1083/jcb.119.5.1183), which provided early insight into the basic physiology of the sarcolemmal membrane and cytoskeletal structure. (worldmusclesociety.org)
Products1