Article
Altered expression of the MCSP/NG2 chondroitin sulfate proteoglycan in collagen VI deficiency.
Molecular and cellular neurosciences - 1 Nov 2005
Petrini Stefania, Tessa Alessandra, Stallcup William B, Sabatelli Patrizia, Pescatori Mario, Giusti Betti, Carrozzo Rosalba, Verardo Margherita, Bergamin Natascha, Columbaro Marta, Bernardini Camilla, Merlini Luciano, Pepe Guglielmina, Bonaldo Paolo, Bertini Enrico
Abstract excerpt
NG2, the rat homologue of the human melanoma chondroitin sulfate proteoglycan (MCSP), is a ligand for collagen VI (COL6). We have examined skeletal muscles of patients affected by Ullrich scleroatonic muscular dystrophy (UCMD), an inherited syndrome caused by COL6 genes mutations. A significant decrease of NG2 immunolabeling was found in UCMD myofibers, as well as in skeletal muscle and cornea of COL6 null-mice....
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.