Anti-Dystrophin/DMD Antibody Picoband® Fluoro647 Conjugated

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) .

E.coli-derived human Dystrophin recombinant protein (Position: H3076-D3404) . Human Dystrophin shares 100% amino acid (aa) sequence identity with mouse Dystrophin.

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. .

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. .

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

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) . .