| Impaired Intracellular Ca(2+) Dynamics, M-Band and Sarcomere Fragility in Skeletal Muscles of Obscurin KO Mice. | Impaired Intracellular Ca(2+) Dynamics, M-Band and Sarcomere Fragility in Skeletal Muscles of Obscurin KO Mice.
Pierantozzi E, Szentesi P, Paolini C, Dienes B, Fodor J, Oláh T, Colombini B, Rassier DE, Rubino EM, Lange S, Rossi D, Csernoch L, Bagni MA, Reggiani C, Sorrentino V., Free PMC Article | 03/12/2022 |
| Deletion of obscurin immunoglobulin domains Ig58/59 leads to age-dependent cardiac remodeling and arrhythmia. | Deletion of obscurin immunoglobulin domains Ig58/59 leads to age-dependent cardiac remodeling and arrhythmia.
Grogan A, Coleman A, Joca H, Granzier H, Russel MW, Ward CW, Kontrogianni-Konstantopoulos A., Free PMC Article | 10/23/2021 |
| Skeletal muscle-specific Obsl1 knockouts show a benign phenotype similar to obscurin knockouts. Only deletion of both proteins and removal of their functional redundancy revealed their roles for sarcolemmal stability and sarcoplasmic reticulum organization. | Murine obscurin and Obsl1 have functionally redundant roles in sarcolemmal integrity, sarcoplasmic reticulum organization, and muscle metabolism.
Blondelle J, Marrocco V, Clark M, Desmond P, Myers S, Nguyen J, Wright M, Bremner S, Pierantozzi E, Ward S, Estève E, Sorrentino V, Ghassemian M, Lange S., Free PMC Article | 04/25/2020 |
| These findings implicate small obscurins in the maintenance of cardiomyocyte size and coupling. | Novel obscurins mediate cardiomyocyte adhesion and size via the PI3K/AKT/mTOR signaling pathway.
Ackermann MA, King B, Lieberman NAP, Bobbili PJ, Rudloff M, Berndsen CE, Wright NT, Hecker PA, Kontrogianni-Konstantopoulos A., Free PMC Article | 05/19/2018 |
| Altogether, these results suggest that obscurin is required for the maintenance of morphological and ultrastructural integrity of skeletal muscle fibers against damage induced by intense mechanical stress and point to the diaphragm as the skeletal muscle most severely affected in obscurin-deficient mice. | Exercise-induced alterations and loss of sarcomeric M-line organization in the diaphragm muscle of obscurin knockout mice.
Randazzo D, Blaauw B, Paolini C, Pierantozzi E, Spinozzi S, Lange S, Chen J, Protasi F, Reggiani C, Sorrentino V., Free PMC Article | 06/10/2017 |
| obscurins are not restricted to striated muscles, but are abundantly expressed in several tissues and organs including brain, skin, kidney, liver, spleen, and lung. | Obscurins: Goliaths and Davids take over non-muscle tissues.
Ackermann MA, Shriver M, Perry NA, Hu LY, Kontrogianni-Konstantopoulos A., Free PMC Article | 01/17/2015 |
| the obscurin kinase domains, SK1 and SK2, are active enzymes with distinct substrate specificities. | The kinase domains of obscurin interact with intercellular adhesion proteins.
Hu LY, Kontrogianni-Konstantopoulos A., Free PMC Article | 07/6/2013 |
| Obscurin, by targeting ankB at the M band, contributes to the organization of subsarcolemma microtubules, localization of dystrophin at costameres, and maintenance of sarcolemmal integrity. | Obscurin is required for ankyrinB-dependent dystrophin localization and sarcolemma integrity.
Randazzo D, Giacomello E, Lorenzini S, Rossi D, Pierantozzi E, Blaauw B, Reggiani C, Lange S, Peter AK, Chen J, Sorrentino V., Free PMC Article | 04/27/2013 |
| the organization of obscurin at different locations in the sarcomere changes during muscle development and that this might affect the interaction with ank1.5. | Localization of ank1.5 in the sarcoplasmic reticulum precedes that of SERCA and RyR: relationship with the organization of obscurin in developing sarcomeres.
Giacomello E, Sorrentino V. | 01/21/2010 |
| the Rho-GEF domain of obscurin interacts with RanBP9 and that both can interact with the N-terminal region of titin to influence the formation of the Z-disk and A/I junction | The rho-guanine nucleotide exchange factor domain of obscurin regulates assembly of titin at the Z-disk through interactions with Ran binding protein 9.
Bowman AL, Catino DH, Strong JC, Randall WR, Kontrogianni-Konstantopoulos A, Bloch RJ., Free PMC Article | 01/21/2010 |
| ANK2 is subject to alternative splicing that gives rise to unique polypeptides with diverse roles in cardiac function. | Obscurin targets ankyrin-B and protein phosphatase 2A to the cardiac M-line.
Cunha SR, Mohler PJ., Free PMC Article | 01/21/2010 |
| Results support a role of obscurin in mediating the subcellular localization of small ankyrin1 isoforms in striated muscle cells. | Molecular interactions with obscurin are involved in the localization of muscle-specific small ankyrin1 isoforms to subcompartments of the sarcoplasmic reticulum.
Armani A, Galli S, Giacomello E, Bagnato P, Barone V, Rossi D, Sorrentino V. | 01/21/2010 |