Altered expression of the MCSP/NG2 chondroitin sulfate proteoglycan in collagen VI deficiency

Stefania Petrini, Alessandra Tessa, William B. Stallcup, Patrizia Sabatelli, Mario Pescatori, Betti Giusti, Rosalba Carrozzo, Margherita Verardo, Natascha Bergamin, Marta Columbaro, Camilla Bernardini, Luciano Merlini, Guglielmina Pepe, Paolo Bonaldo, Enrico Bertini

Research output: Contribution to journalArticle

Abstract

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. In UCMD muscles, truncated NG2 core protein isoforms were detected. However, real-time RT-PCR analysis revealed marked increase in NG2 mRNA content in UCMD muscle compared to controls. We hypothesize that NG2 immunohistochemical and biochemical behavior may be compromised owing to the absence of its physiological ligand. MCSP/NG2 proteoglycan may be considered an important receptor mediating COL6-sarcolemma interactions, a relationship that is disrupted by the pathogenesis of UCMD muscle.

Original languageEnglish
Pages (from-to)408-417
Number of pages10
JournalMolecular and Cellular Neuroscience
Volume30
Issue number3
DOIs
Publication statusPublished - Nov 2005

ASJC Scopus subject areas

  • Molecular Biology
  • Cellular and Molecular Neuroscience
  • Developmental Neuroscience

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