Type: | Family | Name: | Alpha-1-syntrophin |
Description: | Alpha-1-syntrophin (SNTA1) is an adapter protein that binds to and probably organises the subcellular localisation of a variety of membrane proteins. It may link various receptors to the actin cytoskeleton and the extracellular matrix via the dystrophin glycoprotein complex [, , ]. It is partly involved in the pathophysiology of dystrophin-deficient Duchenne muscular dystrophy [].The syntrophin family are intracellular membrane-associated proteins that interact with both ion channels and signalling proteins. As such, syntrophins appear to be important for linking ion channels to intracellular signalling pathways. Syntrophins are concentrated at postsynaptic sites at the neuromuscular junction and may be involved in clustering acetylcholine receptors. In muscle and non-muscle tissues, syntrophin is associated with dystrophin, utrophin, and two homologues of the dystrophin carboxy-terminal region [].The five known syntrophin isoforms share a common domain organisation which includes two tandem pleckstrin homology (PH) domains at the N terminus, a PDZ domain and a C-terminal domain of ~70 amino acids that is unique to syntrophins (syntrophin unique, SU) []. | Short Name: | SNTA1 |