Anti-Utrophin A from rabbit

Utrophin A (UniProt: E9Q6R7) is encoded by the Utrn gene in murine species. Utrophin is a large cytoskeletal protein, which is autosomally-encoded homologue of dystrophin, the protein product of the Duchenne muscular dystrophy (DMD) gene. It displays a sequence homology with dystrophin and possesses many of the protein-binding properties ascribed to dystrophin. In normal skeletal muscle, utrophin is located at the neuromuscular junction and dystrophin predominates at the sarcolemma. During development and in certain myopathies utrophin is also reported to be present at the sarcolemma and this redistribution is associated with higher levels of utrophin. Utrophin is found to co-localize with the acetylcholine receptors at the neuromuscular junctions and may participate in stabilizing the synaptic cytoskeleton. The utrophin mRNA contains two full-length species (named A- and B-utrophin), which are transcribed from different promoters. Hence, utrophin is a composite of A- and B-utrophin and only the A-utrophin is up-regulated in dystrophin-deficient striated muscle. This up-regulation is reported to occur post-transcriptionally. Utrophin A and B differ in their N-termini sequence. (Ref.: Blake, DJ et al. (1996). Brain Pathol. 6(1); 37-47; Weir, AP et al. (2002). J. Biol. Chem. 277 (47); 45285-90).