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PMID:18268005
| Citation |
Stiber, JA, Zhang, ZS, Burch, J, Eu, JP, Zhang, S, Truskey, GA, Seth, M, Yamaguchi, N, Meissner, G, Shah, R, Worley, PF, Williams, RS and Rosenberg, PB (2008) Mice lacking Homer 1 exhibit a skeletal myopathy characterized by abnormal transient receptor potential channel activity. Mol. Cell. Biol. 28:2637-47 |
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| Abstract |
Transient receptor potential (TRP) channels are nonselective cation channels, several of which are expressed in striated muscle. Because the scaffolding protein Homer 1 has been implicated in TRP channel regulation, we hypothesized that Homer proteins play a significant role in skeletal muscle function. Mice lacking Homer 1 exhibited a myopathy characterized by decreased muscle fiber cross-sectional area and decreased skeletal muscle force generation. Homer 1 knockout myotubes displayed increased basal current density and spontaneous cation influx. This spontaneous cation influx in Homer 1 knockout myotubes was blocked by reexpression of Homer 1b, but not Homer 1a, and by gene silencing of TRPC1. Moreover, diminished Homer 1 expression in mouse models of Duchenne's muscular dystrophy suggests that loss of Homer 1 scaffolding of TRP channels may contribute to the increased stretch-activated channel activity observed in mdx myofibers. These findings provide direct evidence that Homer 1 functions as an important scaffold for TRP channels and regulates mechanotransduction in skeletal muscle. |
| Links |
PubMed PMC2293116 Online version:10.1128/MCB.01601-07 |
| Keywords |
Animals; Calcium Signaling; Carrier Proteins/genetics; Carrier Proteins/metabolism; Cells, Cultured; Disease Models, Animal; Gene Deletion; Gene Expression Regulation; Mice; Mice, Knockout; Muscle Contraction; Muscular Dystrophies/genetics; Muscular Dystrophies/pathology; Muscular Dystrophies/physiopathology; Protein Binding; TRPC Cation Channels/genetics; TRPC Cation Channels/metabolism |
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Significance
Annotations
| Gene product | Qualifier | GO ID | GO term name | Evidence Code | with/from | Aspect | Notes | Status |
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