Zobrazeno 1 - 10
of 150
pro vyhledávání: '"James M Ervasti"'
Autor:
Abhijit Dandapat, Benjamin J Perrin, Christine Cabelka, Maria Razzoli, James M Ervasti, Alessandro Bartolomucci, Dawn A Lowe, Michael Kyba
Publikováno v:
PLoS ONE, Vol 11, Iss 3, p e0151467 (2016)
Facioscapulohumeral muscular dystrophy (FSHD) is caused by mutations leading to ectopic expression of the transcription factor DUX4, and encompasses both muscle-related and non-muscle phenotypes. Mouse models bearing this gene represent valuable tool
Externí odkaz:
https://doaj.org/article/8b60ed8b718c45f291e3e516d4464c70
Autor:
Lauren J. Sundby, William M. Southern, Jiao Sun, Xiaobai Patrinostro, Wei Zhang, Jeongsik Yong, James M. Ervasti
Publikováno v:
European Journal of Cell Biology, Vol 103, Iss 2, Pp 151397- (2024)
The cytoplasmic actin proteins, β- and γ-actin, are 99% identical but thought to perform non-redundant functions. The nucleotide coding regions of cytoplasmic actin genes, Actb and Actg1, are 89% identical. Knockout (KO) of Actb by Cre-mediated del
Externí odkaz:
https://doaj.org/article/213c04ad534c416084dde9f9fa09cb67
Publikováno v:
PLoS ONE, Vol 7, Iss 3, p e32970 (2012)
The local translation of β-actin is one mechanism proposed to regulate spatially-restricted actin polymerization crucial for nearly all aspects of neuronal development and function. However, the physiological significance of localized β-actin trans
Externí odkaz:
https://doaj.org/article/6156527fddd142cbb155726b96bdebdc
Publikováno v:
PLoS ONE, Vol 6, Iss 3, p e17768 (2011)
The proper localization of ß-actin mRNA and protein is essential for growth cone guidance and axon elongation in cultured neurons. In addition, decreased levels of ß-actin mRNA and protein have been identified in the growth cones of motor neurons c
Externí odkaz:
https://doaj.org/article/e4c616323678484aad8c8ea766e79875
Publikováno v:
PLoS Genetics, Vol 6, Iss 10, p e1001158 (2010)
Hair cell stereocilia structure depends on actin filaments composed of cytoplasmic β-actin and γ-actin isoforms. Mutations in either gene can lead to progressive hearing loss in humans. Since β-actin and γ-actin isoforms are 99% identical at the
Externí odkaz:
https://doaj.org/article/ea30eb6846ca4c44bfab8ce75806a2d4
Autor:
Kevin J Sonnemann, Hanke Heun-Johnson, Amy J Turner, Kristen A Baltgalvis, Dawn A Lowe, James M Ervasti
Publikováno v:
PLoS Medicine, Vol 6, Iss 5, p e1000083 (2009)
The loss of dystrophin compromises muscle cell membrane stability and causes Duchenne muscular dystrophy and/or various forms of cardiomyopathy. Increased expression of the dystrophin homolog utrophin by gene delivery or pharmacologic up-regulation h
Externí odkaz:
https://doaj.org/article/4fdad23a597c44f7b801c8d249d2427a
Publikováno v:
PLoS ONE, Vol 3, Iss 7, p e2604 (2008)
Alpha-dystrobrevin is a component of the dystrophin-glycoprotein complex (DGC) and is thought to have both structural and signaling roles in skeletal muscle. Mice deficient for alpha-dystrobrevin (adbn(-/-)) exhibit extensive myofiber degeneration an
Externí odkaz:
https://doaj.org/article/f44a2f19637d4a8fb17d315a17b8283d
Publikováno v:
PLoS ONE, Vol 3, Iss 6, p e2419 (2008)
We previously documented a ten-fold increase in gamma(cyto)-actin expression in dystrophin-deficient skeletal muscle and hypothesized that increased gamma(cyto)-actin expression may participate in an adaptive cytoskeletal remodeling response. To expl
Externí odkaz:
https://doaj.org/article/084e035ca0974c39b533919e54933b2c
Autor:
Lisa Duvick, W. Michael Southern, Kellie Benzow, Hillary P. Handler, Jason S. Mitchell, Hannah Kuivinen, Udaya Keerthy Gadiparthi, Praseuth Yang, Alyssa Soles, Carrie Scheeler, Orion Rainwater, Serres Shannah, Erin Larson, Tessa Nichols-Meade, Yun You, Brennon O’Callaghan, Huda Y. Zoghbi, James M. Ervasti, Marija Cvetanovic, Michael D. Koob, Harry T. Orr
Publikováno v:
bioRxiv
Spinocerebellar ataxia type 1 (SCA1) is a fatal neurodegenerative disease caused by an expanded polyglutamine tract in the widely expressed ATXN1 protein. To elucidate anatomical regions and cell types that underlie mutant ATXN1-induced disease pheno
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::097ef39977231952db5f84fa6647e9d2
https://europepmc.org/articles/PMC9934664/
https://europepmc.org/articles/PMC9934664/
Autor:
Lauren J. Sundby, William M. Southern, Katelin M. Hawbaker, Jesús M. Trujillo, Benjamin J. Perrin, James M. Ervasti
Publikováno v:
Molecular Biology of the Cell. 33
Actbc–g Actg1–/– mice expressing only γ-actin from an edited Actbc–g allele have growth and survival defects despite expressing normal levels of γ-actin. Cell and auditory phenotypes associated with Actg1 KO were rescued by γ-actin express
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::64bc16fb64b5cf2a9ba8ec152a563f86
https://doi.org/10.1091/mbc.e22-02-0054
https://doi.org/10.1091/mbc.e22-02-0054