Dystrobrevin
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Dystrobrevin is a protein that binds to dystrophin in the costamere of skeletal muscle cells. In humans, there are at least two isoforms of dystrobrevin, dystrobrevin alpha and dystrobrevin beta.
dystrobrevin, alpha | |||||||
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Identifiers | |||||||
Symbol | DTNA | ||||||
NCBI gene | 1837 | ||||||
HGNC | 3057 | ||||||
OMIM | 601239 | ||||||
RefSeq | NM_032981 | ||||||
UniProt | Q9Y4J8 | ||||||
Other data | |||||||
Locus | Chr. 18 q12 | ||||||
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dystrobrevin, beta | |||||||
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Identifiers | |||||||
Symbol | DTNB | ||||||
NCBI gene | 1838 | ||||||
HGNC | 3058 | ||||||
OMIM | 602415 | ||||||
RefSeq | NM_033147 | ||||||
UniProt | O60941 | ||||||
Other data | |||||||
Locus | Chr. 2 p24 | ||||||
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Dystrobrevins are members of dystrophin-related protein family which are thought to play an important role in intracellular signal transduction and provide a membrane scaffold in muscle. Defects in dystrobrevins and their associated proteins cause a range of neuromuscular diseases such as muscular dystrophies. Dystrobrevin was first identified by isolating from the electric organ of the electric ray Torpedo californica.[1] It is a phosphoprotein, which weights 87 kDa, associated with the postsynaptic membrane at the cytoplasmic face.[2][3] Dystrobrevin proteins have been said to participates in the formation and stability of synapses because it copurifies with acetylcholine receptors from Torpedo electric organ membranes.[4]
In 1997, an experiment was done using the yeast two-hybrid model to identify protein-protein interaction between dystrobrevin and dystrophin-associated protein complex (DPC). The evidence suggested that dystrobrevin works as a motor protein receptor that might play an important role in the transport of components of the dystrophin-associated protein complex to specific intracellular sites.[5] The DPC is expressed in both muscle and non-muscle tissues. It works as a mechanical component of cells and a dynamic multifunctional structure that can serve as a scaffold for signaling molecules.[6] The dystrophin-associated proteins can be divided into three groups depending on their cellular localization: extracellular, transmembrane, and cytoplasmic. Dystrobrevin protein is a part of the cytoplasmic complex and an intracellular protein that binds directly to dystrophin.
In invertebrates, dystrobrevin is present as a single protein, while in vertebrates, there are two isoforms, a-dystrobrevin (DTNA) and β-dystrobrevin (DTNB).[7] Each dystrobrevin isoform has a unique structure with carboxyl termini and sequence homology with the cysteine-rich carboxyl-terminal region of dystrophin. This region of similarity can be divided into several functional domains such as two coiled-coil regions, two EF hands or a ZZ-type zinc finger.[6]