The Rho-Guanine Nucleotide Exchange Factor Domain of Obscurin Regulates Assembly of Titin at the Z-Disk through Interactions with Ran Binding Protein 9

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Population

Maturing myofibrils (striated muscle)

Design

Preclinical

Authors

Discussion

Cellular disruption of Z-disk assembly; hypothesis-generating for RanBP9-obscurin-titin roles in cardiomyopathy.

Population

Maturing myofibrils (striated muscle)

Intervention

Adenoviral overexpression of the Rho-GEF domain of obscurin and the binding region of RanBP9

Outcome

Incorporation of titin into developing Z-disks and structure of the Z-disk and Z/I junctionsurrogate

The Rho-GEF domain of obscurin and its novel ligand RanBP9 interact with the N-terminal region of titin to regulate sarcomere assembly at the Z-disk.

Bowman et al. (2008) studied this question.

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