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Published online 12 June 2006. doi:10.1083/jcb.200603119
The Rockefeller University Press, 0021-9525 $8.00
JCB, Volume 173, Number 6, 905-916
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Article

 Nebulin-deficient mice exhibit shorter thin filament lengths and reduced contractile function in skeletal muscle

Marie-Louise Bang1, Xiaodong Li1, Ryan Littlefield5, Shannon Bremner3,4, Andrea Thor2, Kirk U. Knowlton1, Richard L. Lieber3,4, and Ju Chen1

1 Department of Medicine, 2 National Center for Microscopy and Imaging Research, 3 Department of Orthopaedic Surgery, and 4 Department of Bioengineering, Veterans Affairs Medical Center, University of California, San Diego, La Jolla, CA 92093
5 Center for Cell Dynamics, Friday Harbor Laboratories, University of Washington, Friday Harbor, WA 98250

Correspondence to Ju Chen: juchen{at}ucsd.edu

Nebulin is a giant modular sarcomeric protein that has been proposed to play critical roles in myofibrillogenesis, thin filament length regulation, and muscle contraction. To investigate the functional role of nebulin in vivo, we generated nebulin-deficient mice by using a Cre knock-in strategy. Lineage studies utilizing this mouse model demonstrated that nebulin is expressed uniformly in all skeletal muscles. Nebulin-deficient mice die within 8–11 d after birth, with symptoms including decreased milk intake and muscle weakness. Although myofibrillogenesis had occurred, skeletal muscle thin filament lengths were up to 25% shorter compared with wild type, and thin filaments were uniform in length both within and between muscle types. Ultrastructural studies also demonstrated a critical role for nebulin in the maintenance of sarcomeric structure in skeletal muscle. The functional importance of nebulin in skeletal muscle function was revealed by isometric contractility assays, which demonstrated a dramatic reduction in force production in nebulin-deficient skeletal muscle.

X. Li's present address is Division of Cardiothoracic Surgery, University of California, San Diego, La Jolla, CA 92103.

Abbreviations used in this paper: EDL, extensor digitorum longus; ES, embryonic stem; PCSA, physiological cross-sectional area; SH3, Src homology 3; TA, tibialis anterior; TEM, transmission EM; VL, vastus lateralis.


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