Muscle interstitial fibroblasts are the main source of collagen VI synthesis in skeletal muscle:: Implications for congenital muscular dystrophy types Ullrich and Bethlem

被引:108
作者
Zou, Yaqun [1 ]
Zhang, Rui-Zhu [2 ]
Sabatelli, Patrizia [3 ]
Chu, Mon-Li [2 ]
Bonnemann, Carsten G. [1 ]
机构
[1] Childrens Hosp Philadelphia, Abramson Res Ctr, Div Neurol, Philadelphia, PA 19104 USA
[2] Thomas Jefferson Univ, Dept Dermatol & Cutaneous Biol, Philadelphia, PA 19107 USA
[3] CNR, Unit Bologna, IGM, Bologna, Italy
关键词
Bethlem myopathy; collagen VI; congenital muscular dystrophy type Ullrich; interstitial fibroblasts;
D O I
10.1097/nen.0b013e3181634ef7
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Mutations in the extracellular matrix molecule collagen VI underlie the congenital muscular dystrophy types Ullrich and Bethlem. Establishing the origin of collagen VI in muscle is important for understanding the pathophysiology of these diseases and for developing future treatment approaches involving cell-specific delivery. Because the cells that produce collagen VI cannot be identified by histologic analysis, we examined the production of collagen VI in pure cultures of primary myogenic cells and muscle interstitial fibroblasts from limb muscle of neonatal mice. Immunofluorescence staining and Western blot analysis revealed secretion and matrix deposition of collagen VI by interstitial fibroblasts but not by myogenic cells in vitro. Using Northern blot and real-time reverse-transcriptase-polymerase chain reaction analysis for the collagen VI genes col6a1, col6a2, col6a3, transcript levels for the 3 mRNAs were high in interstitial fibroblasts, whereas in primary myogenic cells, they were indistinguishable from background. Furthermore, retention of mutant collagen VI in muscle from 3 patients with collagen VI mutation was identified in interstitial fibroblastic cells but not in their myofibers. These results suggest that interstitial fibroblasts but not myogenic cells contribute significantly to the deposition of collagen VI in the extracellular matrix in skeletal muscle and imply major roles of this cell type and the extracellular matrix in the pathogenesis of these diseases.
引用
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页码:144 / 154
页数:11
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