- Catalog number
- PB9276
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- Dystrophin, also known as DMD, is a rod-shaped cytoplasmic protein, and a vital part of a protein complex that connects the cytoskeleton of a muscle fiber to the surrounding extracellular matrix through the cell membrane. It is mapped to Xp21.2-p21.1. This complex is variously known as the costamere or thedystrophin-associated protein complex. Many muscle proteins, such as α-dystrobrevin, syncoilin, synemin, sarcoglycan, dystroglycan, and sarcospan, colocalize with dystrophin at the costamere. Dystrophin is a protein located between the sarcolemma and the outermost layer of myofilaments in the muscle fiber (myofiber) . It is a cohesive protein, linking actin filaments to another support protein that resides on the inside surface of each muscle fiber’s plasma membrane (sarcolemma) .
- Synonyms
- Dystrophin; DMD
- Gene Name
- DMD
- Gene ID
- 1756
- UniProt
- P11532
- Host
- Rabbit
- Reactivity
- Human, Mouse, Rat
- Cross Reactivity
- No cross-reactivity with other proteins
- Immunogen
- E.coli-derived human Dystrophin recombinant protein (Position: H3076-D3404) . Human Dystrophin shares 100% amino acid (aa) sequence identity with mouse Dystrophin.
- Clonality
- Polyclonal
- Tissue Specificity
- Expressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Expressed in brain, muscle, kidney, lung and testis. Isoform 5 is expressed in heart, brain, liver, testis and hepatoma cells. Most tissues contain transcripts of multiple isoforms, however only isoform 5 is detected in heart and liver. .
- Applications
- Flow Cytometry, IHC, IHC-F, ICC, WB
- Field of Research
- Biochemicals, Chemical Type, Signal Transduction
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- 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. .
- References & Citations
- 1. Tennyson CN, Klamut HJ, Worton RG (1995) . The human dystrophin gene requires 16 hours to be transcribed and is cotranscriptionally spliced. Nature Genetics 9 (2) : 184–90. 2. García-Pelagio KP, Bloch RJ, Ortega A, González-Serratos H (2011) . Biomechanics of the sarcolemma and costameres in single skeletal muscle fibers from normal and dystrophin- null mice. J Muscle Res Cell Motil 31 (5-6) : 323–336. 3. Strachan T and Read AP, 1999. Human molecular genetics, BIOS Scientific, New York, USA
- Storage Conditions
- Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 426750 MW
- Observed Molecular Weight
- 427 kDa
- Specificity
- No cross reactivity with other proteins.
- Applications Notes
- 6
- Gene Name Synonym
- Dystrophin
- Subcellular Location
- 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 the presence of ANK2 (By similarity) . .
- Sequence Similarities
- Contains 2 CH (calponin-homology) domains.
- Protein Name
- Dystrophin
- Isotype
- Rabbit IgG
- Contents
- Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.