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Rabbit Anti-Utrophin/FITC Conjugated antibody
background:
Dystrophin and utrophin are related structural, Actin-binding proteins that are involved in anchoring the cytoskeleton to the plasma membrane. Dystrophin is the protein product of the Duchenne/Becker muscular dystrophy gene. Dystrophin expression is found in muscle and brain tissues, where it is localized to the inner surface of the plasma membrane. It has been speculated that alternative splicing of the carboxy terminus allows dystrophin to interact with a variety of proteins. Research has shown that the loss of dystrophin-associated proteins in Duchenne afflicted muscle is due to the absence of dystrophin rather than to muscle degradation and that the lack of dystrophin results in the loss of linkage between the cytoskeleton and the extracellular matrix. Evidence suggests that the upregulation of utrophin can reduce the dystrophic pathology.
Function:
May play a role in anchoring the cytoskeleton to the plasma membrane.
Subunit:
Interacts with the syntrophins SNTA1; SNTB1 and SNTB2. Interacts with SYNM. Interacts (via its WWW and ZZ domains) with DAG1 (via the PPXY motif of betaDAG1); the interaction is inhibited by the tyrosine phosphorylation of the PPXY motif of DAG1.
Subcellular Location:
Cell junction > synapse > postsynaptic cell membrane. Cytoplasm > cytoskeleton. Neuromuscular junction.
Tissue Specificity:
Muscle.
Similarity:
Contains 2 CH (calponin-homology) domains.
Contains 20 spectrin repeats.
Contains 1 WW domain.
Contains 1 ZZ-type zinc finger.
Database links:
Entrez Gene: 7402 Human
Omim: 12848 Human
SwissProt: P46939 Human
Unigene: 133135 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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