What does dystrophin glycoprotein complex do?
The dystrophin-glycoprotein complex (DGC) links the muscle cytoskeleton to the extracellular matrix and is responsible for force transduction and protects the muscle fibres from contraction induced damage. Mutations in components of the DGC are responsible for muscular dystrophies and congenital myopathies.
What is the dystroglycan complex?
The dystroglycan complex contains the transmembrane protein β-dystroglycan and its interacting extracellular mucin-like protein α-dystroglycan. In skeletal muscle fibers, the dystroglycan complex plays an important structural role by linking the cytoskeletal protein dystrophin to laminin in the extracellular matrix.
What other protein or protein complexes does dystrophin bind?
Dystrophin is a multidomain protein that links the actin cytoskeleton to laminin in the extracellular matrix through the dystrophin associated protein (DAP) complex. The COOH-terminal domain of dystrophin binds to two components of the DAP complex, syntrophin and dystrobrevin.
What is dystrophin and why is it important?
Dystrophin is a protein found in muscle cells. It is one of a group of proteins that work together to strengthen muscle fibers and protect them from injury as muscles contract and relax.
Is Nebulin a contractile protein?
Nebulin is an actin-binding protein which is localized to the thin filament of the sarcomeres in skeletal muscle. It is a very large protein (600–900 kDa) and binds as many as 200 actin monomers….Nebulin.
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What is another analogy for how dystrophin works?
This link stabilizes muscle fibers during muscle contraction (exercise). I like to explain the function of dystrophin by the analogy of an anchor (the skeleton of the muscle) and a boat (the protective layer outside), where dystrophin is the rope that connects the two.
What does dystroglycan bind to?
On the intracellular side, the dystroglycan complex will bind to the proteins dystrophin or utrophin. In turn, dystrophin and utrophin will bind to the actin cytoskeleton.
Is dystrophin a structural protein?
In addition to its structural role, dystrophin complex is also thought to mediate cellular signaling such as mechanical force transduction and cell adhesion. This review will focus on dystrophin and its associated proteins.
What does dystrophin do in normal muscle?
Normal Function In skeletal and cardiac muscles, dystrophin is part of a group of proteins (a protein complex) that work together to strengthen muscle fibers and protect them from injury as muscles contract and relax.
What is the main function of dystrophin?
Which is the largest human gene?
human dystrophin gene
The largest known gene is the human dystrophin gene, which has 79 exons spanning at least 2,300 kilobases (kb).