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 language | English |
|---|---|
| Pages (from-to) | 408-417 |
| Number of pages | 10 |
| Journal | Molecular and Cellular Neuroscience |
| Volume | 30 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - Nov 2005 |
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ASJC Scopus subject areas
- Molecular Biology
- Cellular and Molecular Neuroscience
- Developmental Neuroscience
Cite this
Altered expression of the MCSP/NG2 chondroitin sulfate proteoglycan in collagen VI deficiency. / 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.
In: Molecular and Cellular Neuroscience, Vol. 30, No. 3, 11.2005, p. 408-417.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Altered expression of the MCSP/NG2 chondroitin sulfate proteoglycan in collagen VI deficiency
AU - Petrini, Stefania
AU - Tessa, Alessandra
AU - Stallcup, William B.
AU - Sabatelli, Patrizia
AU - Pescatori, Mario
AU - Giusti, Betti
AU - Carrozzo, Rosalba
AU - Verardo, Margherita
AU - Bergamin, Natascha
AU - Columbaro, Marta
AU - Bernardini, Camilla
AU - Merlini, Luciano
AU - Pepe, Guglielmina
AU - Bonaldo, Paolo
AU - Bertini, Enrico
PY - 2005/11
Y1 - 2005/11
N2 - 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.
AB - 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.
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UR - http://www.scopus.com/inward/citedby.url?scp=26244435554&partnerID=8YFLogxK
U2 - 10.1016/j.mcn.2005.08.005
DO - 10.1016/j.mcn.2005.08.005
M3 - Article
C2 - 16169245
AN - SCOPUS:26244435554
VL - 30
SP - 408
EP - 417
JO - Molecular and Cellular Neurosciences
JF - Molecular and Cellular Neurosciences
SN - 1044-7431
IS - 3
ER -