This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.Nebulin is a giant modular skeletal muscle specific protein (500-900 kDa), which extends along the thin filament in muscle and thought to be a regulator of thin filament length. In heart, a small nebulin-like molecule, nebulette is associated with thin filaments of cardiac myofibrils, but extends only into the central I-band region. We have generated two mouse models deficient for nebulin and nebulette, respectively. To study the structural role of these two molecules and to determine whether nebulin is indeed a regulator of thin filament length we are in the process of performing TEM and immunolabeling studies.
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