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- W2912884581 abstract "Nebulin is a giant, modular actin binding protein that spans along the thin filament in skeletal muscle. Nebulin consists of numerous tandem copies of 38.5 nm ‘super-repeats’ that correspond to the arrangement of troponin and tropomyosin on the thin filament. Besides being known as a thin filament stabilizer, nebulin has been speculated to act as a thin filament ruler in skeletal muscle, yet little is known about nebulin's direct influence on actin length or its mechanism of action. Here we developed two novel mouse models in which nebulin super-repeats 9-11 are deleted (NebΔSR9-11) or duplicated (NebdupSR9-11), respectively. Mice of either model are viable and develop without muscle weight deficits. Super-resolution microscopy (SR-SIM) and immunoelectron microscopy on extensor digitorum longus (EDL) muscle revealed that the N-terminus of nebulin, together with tropomodulin (Tmod) localizes at the thin filament pointed end. Furthermore, nebulin and thin filaments were found to be shorter by ∼115 nm in NebΔSR9-11 and longer by ∼115 nm in NebdupSR9-11 EDL. Compound heterozygous animals (NebΔSR9-11,dupSR9-11) express both the shorter and the longer nebulin close to a ratio of 1:1 and the difference in their lengths is ∼230 nm. Tmod localization suggests a bimodal thin filament length distribution in NebΔSR9-11,dupSR9-11 EDL muscle fibers according to the shorter and longer nebulin. Functional studies on skinned EDL fibers revealed leftward shift of the descending limb of the force-sarcomere length relationship in NebΔSR9-11 and rightward shift in NebdupSR9-11 consistent with their altered thin filament lengths. We conclude that nebulin rules the thin filaments in EDL muscle with each of nebulin's super-repeats being responsible for a quantal 38 nm thin filament length. Studies on other muscle types are currently ongoing." @default.
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- W2912884581 date "2019-02-01" @default.
- W2912884581 modified "2023-09-27" @default.
- W2912884581 title "Experimentally Varying the Number of Super-Repeats in the Neb Gene of the Mouse - Assessing the Role of Nebulin in Thin Filament Length Regulation" @default.
- W2912884581 doi "https://doi.org/10.1016/j.bpj.2018.11.2964" @default.
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