Anti-Dystrophin

0.5mg/ml if reconstituted with 0.2ml sterile DI water. 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 alpha-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 fiberÆs plasma membrane (sarcolemma). Protein 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. [The UniProt Consortium]