Typical Testing Data/Standard Curve (for reference only)
Mouse Dystrophin ELISA Kit | Dmd elisa kit
Mouse Dystrophin ELISA Kit
Typical Testing Data/Standard Curve (for reference only)
Typical Testing Data/Standard Curve (for reference only)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers in skeletal and cardiac muscles. The encoded protein, dystrophin, is part of the dystrophin-glycoprotein complex, which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. This protein is required for proper development and organization of myofibers as contractile units in striated muscles. Mutations in the human gene cause Duchenne and Becker Muscular Dystrophies and a form of heart disease called DMD-associated dilated cardiomyopathy. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined. [provided by RefSeq, Sep 2015]
Uniprot Description
dystrophin: Anchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. 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. Also implicated in signaling events and synaptic transmission. Defects in DMD are the cause of Duchenne muscular dystrophy (DMD). DMD is the most common form of muscular dystrophy; a sex-linked recessive disorder. It typically presents in boys aged 3 to 7 year as proximal muscle weakness causing waddling gait, toe-walking, lordosis, frequent falls, and difficulty in standing up and climbing up stairs. The pelvic girdle is affected first, then the shoulder girdle. Progression is steady and most patients are confined to a wheelchair by age of 10 or 12. Flexion contractures and scoliosis ultimately occur. About 50% of patients have a lower IQ than their genetic expectations would suggest. There is no treatment. Defects in DMD are the cause of Becker muscular dystrophy (BMD). BMD resembles DMD in hereditary and clinical features but is later in onset and more benign. Defects in DMD are a cause of cardiomyopathy dilated X- linked type 3B (CMD3B); also known as X-linked dilated cardiomyopathy (XLCM). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. 6 isoforms of the human protein are produced by alternative splicing.
Protein type: Motility/polarity/chemotaxis; Cytoskeletal
Cellular Component: cell junction; cell surface; cell-matrix junction; costamere; cytoplasm; cytoskeleton; dystrophin-associated glycoprotein complex; filopodium; filopodium membrane; lipid raft; membrane; nucleus; plasma membrane; postsynaptic membrane; protein complex; sarcolemma; synapse; Z disc
Molecular Function: actin binding; metal ion binding; myosin binding; nitric-oxide synthase binding; protein binding; structural constituent of cytoskeleton; structural constituent of muscle; vinculin binding; zinc ion binding
Biological Process: cardiac muscle contraction; cellular protein complex assembly; establishment of blood-nerve barrier; muscle attachment; muscle development; muscle fiber development; muscle maintenance; myotube cell development; negative regulation of peptidyl-serine phosphorylation; neurotransmitter receptor metabolic process; nucleus localization; olfactory nerve structural organization; peptide biosynthetic process; positive regulation of cell-matrix adhesion; positive regulation of neuron differentiation; regulation of gene expression; regulation of heart rate; regulation of membrane potential; regulation of skeletal muscle contraction; regulation of skeletal muscle contraction via regulation of the release of sequestered calcium ion; regulation of transcription, DNA-dependent; skeletal muscle development
Research Articles on Dmd
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Product Notes
The Mouse Dmd dmd (Catalog #AAA2882145) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA2882145 ELISA Kit recognizes Mouse Dmd. It is sometimes possible for the material contained within the vial of "Dystrophin, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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