Ozz, A Protein That Regulates Development and Function of Muscle Cells (SJ-99-0010)



Technology Name
(St. Jude Reference #)
Ozz, A Protein That Regulates Development and Function of Muscle Cells (SJ-99-0010)
Description Ozz is a muscle-specific adapter protein of an E3 ligase complex that ubiquitinates ß-catenin and may target ß-catenin and MyHCemb for degradation during myogenesis. Deregulation of Ozz activity in developing and/or differentiating muscle fibers may influence cellular homeostasis and in turn result in heart or skeletal muscle myopathies. The Ozz gene overlaps with the transcriptional unit of the protective protein/cathepsin A (PPCA) gene, which is mutated in the human lysosomal disease galactosialidosis. It is possible that a mutation in the PPCA gene may affect expression of Ozz.
Keywords Muscle, ubiquitin
Granted Patents or Published Applications U.S. Patent Nos. 7,074,908 and 7,425,615
Related Scientific References Tommaso Nastasi, et. al., "Ozz-E3, A Muscle-Specific Ubiquitin Ligase, Regulates β-Catenin Degradation during Myogenesis" Developmental Cell, Vol 6, 269-282, February 2004.
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Last update: September 2008