Anti-Dystrophin Antibody (RMAB-0250533)
Cat. No.: RMAB-0250533
Category: Primary Antibodies
INQUIRY
100 μL
Customer Size
Rabbit monoclonal to Dystrophin
Product Features
| Isotype | IgG |
|---|---|
| Clonality | Monoclonal |
| Host Species | Rabbit |
| Clone Number | EPR23336-129 |
| Form | Liquid |
| Purity | Protein A purified |
| Species Reactivity | Human, Mouse, Rat |
| Immunogen | Recombinant fragment. |
| Applications | IP, IHC-Fr, IHC-P, mIHC, WB |
| Key Features | Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply; Rabbit monoclonal to Dystrophin; Suitable for: IP, IHC-Fr, IHC-P, mIHC, WB; Reacts with: Mouse, Rat, Human |
Target Information
| Target Symbol | DMD |
|---|---|
| Target Name | Dystrophin |
| UniProt ID | P11532 |
| Cellular Localization | Cell membrane > sarcolemma. Cytoplasm > cytoskeleton. |
| Function | 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. |
| Involvement in Disease | 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 1 or 12. Flexion contractures and scoliosis ultimately occur. About 5% 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. |
| Sequence Similarities | Contains 2 CH (calponin-homology) domains. Contains 22 spectrin repeats. Contains 1 WW domain. Contains 1 ZZ-type zinc finger. |
Storage & Shipping
| Storage Buffer | Preservative: 0.01% Sodium azide; Constituents: 59.94% PBS, 40% Glycerol, 0.05% BSA |
|---|---|
| Storage & Shipping | Shipped at 4°C. Store at 4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. |
For research use only. Not for clinical use.
