Anti-Dystrophin Antibody (Monoclonal, MANDYS8)
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Application
| WB, IHC-P |
|---|---|
| Primary Accession | P11530 |
| Host | Mouse |
| Isotype | Mouse IgG2b |
| Reactivity | Human, Mouse, Rat |
| Clonality | Monoclonal |
| Format | Lyophilized |
| Description | Mouse IgG monoclonal antibody for Dystrophin, dystrophin (DMD) detection. Tested with WB, IHC-P in Human;mouse;rat;rabbit. No cross reactivity with other proteins. |
| Reconstitution | Add 1ml of PBS buffer will yield a concentration of 100ug/ml. |
| Gene ID | 24907 |
|---|---|
| Other Names | Dystrophin, Dmd |
| Calculated MW | 425828 Da |
| Application Details | Immunohistochemistry(Paraffin-embedded Section), 2-4 µg/ml, Human, mouse, rabbit, rat, By Heat Western blot, 1-2 µg/ml, Human, mouse, rabbit, rat |
| Subcellular Localization | Cell membrane, sarcolemma ; Peripheral membrane protein ; Cytoplasmic side . Cytoplasm, cytoskeleton . Cell junction, synapse, postsynaptic cell membrane . In muscle cells, sarcolemma localization requires the presence of ANK2, while localization to costameres requires the presence of ANK3. Localizes to neuromuscular junctions (NMJs). In adult muscle, NMJ localization depends upon ANK2 presence, but not in newborn animals. . |
| Tissue Specificity | Strongly expressed in skeletal muscle and weak expression observed in newborn brain which increases in adult brain. . |
| Source | Eukaryota |
| Protein Name | Dystrophin |
| Contents | Mouse ascites fluid, 1.2% sodium acetate, 2mg BSA, with 0.01mg NaN3 as preservative. |
| Clone Names | MANDYS8 |
| Immunogen | Recombinant human dystrophin fragment. |
| Purification | Ascites |
| Cross Reactivity | No cross reactivity with other proteins |
| Storage | At -20˚C for one year. After r˚Constitution, at 4˚C for one month. It˚Can also be aliquotted and stored frozen at -20˚C for a longer time.Avoid repeated freezing and thawing. |
| Sequence Similarities | Contains 2 CH (calponin-homology) domains. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | Dmd |
|---|---|
| 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. |
| Cellular Location | Cell membrane, sarcolemma {ECO:0000250|UniProtKB:P11531}; Peripheral membrane protein {ECO:0000250|UniProtKB:P11531}; Cytoplasmic side {ECO:0000250|UniProtKB:P11531}. Cytoplasm, cytoskeleton {ECO:0000250|UniProtKB:P11531}. Postsynaptic cell membrane {ECO:0000250|UniProtKB:P11531}. Note=In muscle cells, sarcolemma localization requires the presence of ANK2, while localization to costameres requires the presence of ANK3. Localizes to neuromuscular junctions (NMJs). In adult muscle, NMJ localization depends upon ANK2 presence, but not in newborn animals. {ECO:0000250|UniProtKB:P11531} |
| Tissue Location | Strongly expressed in skeletal muscle and weak expression observed in newborn brain which increases in adult brain |
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
Dystrophin(DMD) gene has 79 exons spanning at least 2,300 kb(2.3 Mb). The C terminus of the dystrophin protein is encoded by a highly conserved, alternatively spliced region of the gene. beta-dystroglycan binding activity is expressed by the dystrophin fragment spanning amino acids 3026-3345 containing the ZZ domain. DMD transcript is formed by at least 60 exons; the first half of the transcript is formed by a minimum of 33 exons spanning nearly 1000 kb, and the remaining portion has at least 27 exons that may spread over a similar distance. Dystrophin gene is expressed at a higher level in primary cultures of neuronal cells than in astro-glial cells derived from adult mouse brain. overexpression of dystrophin prevents the development of the abnormal mechanical properties associated with dystrophic muscle without causing deleterious side effects.
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