title: Obscurin-like 1, OBSL1, is a novel cytoskeletal protein related to obscurin.
abstract: Cytoskeletal adaptor proteins serve vital functions in linking the internal cytoskeleton of cells to the cell membrane, particularly at sites of cell-cell and cell-matrix interactions. The importance of these adaptors to the structural integrity of the cell is evident from the number of clinical disease states attributable to defects in these networks. In the heart, defects in the cytoskeletal support system that surrounds and supports the myofibril result in dilated cardiomyopathy and congestive heart failure. In this study, we report the cloning and characterization of a novel cytoskeletal adaptor, obscurin-like 1 (OBSL1), which is closely related to obscurin, a giant structural protein required for sarcomere assembly. Multiple isoforms arise from alternative splicing, ranging in predicted molecular mass from 130 to 230 kDa. OBSL1 is located on human chromosome 2q35 within 100 kb of SPEG, another gene related to obscurin. It is expressed in a broad range of tissues and localizes to the intercalated discs, to the perinuclear region, and overlying the Z lines and M bands of adult rat cardiac myocytes. Further characterization of this novel cytoskeletal linker will have important implications for understanding the physical interactions that stabilize and support cell-matrix, cell-cell, and intracellular cytoskeletal connections.
authors.affiliation: Department of Pediatrics and Communicable Diseases, University of Michigan, L1242 Women's Hospital/Box 0204, 1500 E. Medical Center Drive, Ann Arbor, MI 48109-0204, USA.
authors.forename: Sarah B
authors.initials: SB
authors.lastname: Geisler
authors.forename: Dustin
authors.initials: D
authors.lastname: Robinson
authors.forename: Maria
authors.initials: M
authors.lastname: Hauringa
authors.forename: Maide O
authors.initials: MO
authors.lastname: Raeker
authors.forename: Andrei B
authors.initials: AB
authors.lastname: Borisov
authors.forename: Margaret V
authors.initials: MV
authors.lastname: Westfall
authors.forename: Mark W
authors.initials: MW
authors.lastname: Russell
mesh_terms: D000818:Animals; D015153:Blotting, Western; D002874:Chromosome Mapping; D002889:Chromosomes, Human, Pair 2; D003001:Cloning, Molecular; D003598:Cytoskeletal Proteins / Q000032:analysis / Q000235:genetics* / Q000502:physiology; D020662:Guanine Nucleotide Exchange Factors / Q000235:genetics; D006801:Humans; D009124:Muscle Proteins / Q000235:genetics; D032383:Myocytes, Cardiac / Q000737:chemistry; D009928:Organ Specificity; D017346:Protein Serine-Threonine Kinases; D051381:Rats; D064067:Rho Guanine Nucleotide Exchange Factors
chemical_list: D003598:Cytoskeletal Proteins; D020662:Guanine Nucleotide Exchange Factors; D009124:Muscle Proteins; C522000:OBSL1 protein, human; C522001:OBSL1 protein, rat; D064067:Rho Guanine Nucleotide Exchange Factors; C578224:OBSCN protein, human; D017346:Protein Serine-Threonine Kinases