Purinergic receptors in skeletal muscles in health and in muscular dystrophy
Krasowska, E.żb.; Róg, J.; Sinadinos, A.; Young, C.N.J.; Górecki, D.C.; Zabłocki, K.
Postepy Biochemii 60(4): 483-489
2014
ISSN/ISBN: 0032-5422 PMID: 25807827 Document Number: 675539
The P2 purinergic (nucleotide) receptor super-family comprises of two families of protein. The P2X, which are channel-forming ionotropic receptors and the P2Y metabotropic receptors activating G protein-mediated signalling pathways. Members of both groups have been identified in skeletal muscle cells at different stages of differentiation. It is well documented that sequential expression and down-regulation of particular P2 receptors on the surface of sarcolemma is closely associated with muscle maturation during embryogenesis and postnatal growth. P2 receptors are also involved in muscle regeneration following injury. Moreover, enhanced expression of specific purinergic receptors together with increased availability of extracellular ATP in dystrophic muscles are important elements of the dys- trophic pathophysiology considerably increasing severity.