title: Obscurins: unassuming giants enter the spotlight.
abstract: Discovered about a decade ago, obscurin (~720 kDa) is a member of a family of giant proteins expressed in striated muscle that are essential for normal muscle function. Much of what we understand about obscurin stems from its functions in cardiac and skeletal muscle. However, recent evidence has indicated that variants of obscurin ("obscurins") are expressed in diverse cell types, where they contribute to distinct cellular processes. Dysfunction or abrogation of obscurins has also been implicated in the development of several pathological conditions, including cardiac hypertrophy and cancer. Herein, we present an overview of obscurins with an emphasis on novel findings that demonstrate their heretofore-unsuspected importance in cell signaling and disease progression.
authors.affiliation: Department of Biochemistry and Molecular Biology, University of Maryland School of Medicine, Baltimore, MD 21201, USA.
authors.forename: Nicole A
authors.initials: NA
authors.lastname: Perry
authors.forename: Maegen A
authors.initials: MA
authors.lastname: Ackermann
authors.forename: Marey
authors.initials: M
authors.lastname: Shriver
authors.forename: Li-Yen R
authors.initials: LY
authors.lastname: Hu
authors.forename: Aikaterini
authors.initials: A
authors.lastname: Kontrogianni-Konstantopoulos
mesh_terms: D000818:Animals; D024741:Cardiomyopathy, Hypertrophic, Familial / Q000235:genetics; D006801:Humans; D024510:Muscle Development; D018482:Muscle, Skeletal / Q000378:metabolism; D020125:Mutation, Missense; D009369:Neoplasms / Q000235:genetics; D009928:Organ Specificity; D060066:Protein Interaction Maps; D017346:Protein Serine-Threonine Kinases; D064067:Rho Guanine Nucleotide Exchange Factors / Q000502:physiology*
chemical_list: D064067:Rho Guanine Nucleotide Exchange Factors; C578224:OBSCN protein, human; D017346:Protein Serine-Threonine Kinases
keywords: OBSCN; UNC-89; breast cancer; cardiac hypertrophy; obscurin; striated muscle