期刊
NEUROMUSCULAR DISORDERS
卷 18, 期 5, 页码 413-422出版社
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.nmd.2008.03.010
关键词
telethonin; myogenic regulatory factors; muscular dystrophy
资金
- NIAMS NIH HHS [T32 AR048523-02] Funding Source: Medline
Null mutation of titin-cap (TCAP) causes limb-girdle muscular dystrophy type 2G (LGMD2G). LGMD2G patients develop muscle atrophy, and lose the ability to walk by their third decade. Previous findings suggest that TCAP regulates myostatin, a key regulator of muscle growth. We tested the hypothesis that TCAP knockdown with RNA interference will lead to differential expression of genes involved in muscle proliferation and differentiation, impairing muscle cell growth. mRNA from cultured cells treated with TCAP siRNA duplex constructs was analyzed using Northern blots and real-time RT-PCR. siRNA treatment decreased TCAP mRNA expression in differentiating muscle cells. Significant (p < 0.05) decreases in mRNA were observed for myogenic regulatory factors. siRNA treatment also prevented development of the normal phenotype of muscle cells. Our findings suggest that TCAP knockdown with RNA interference alters normal muscle cell differentiation. (c) 2008 Elsevier B.V. All rights reserved.
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