scholarly journals Proteomics, bioinformatics and targeted gene expression analysis reveals up-regulation of cochlin and identifies other potential biomarkers in the mouse model for deafness in usher syndrome type 1F

2010 ◽  
Vol 19 (8) ◽  
pp. 1515-1527 ◽  
Author(s):  
Mark R. Chance ◽  
Jinsook Chang ◽  
Shuqing Liu ◽  
Giridharan Gokulrangan ◽  
Daniel H.-C. Chen ◽  
...  
2019 ◽  
Vol 62 (1) ◽  
pp. 036-046 ◽  
Author(s):  
Ran Nagahara ◽  
Tomomitsu Matono ◽  
Takaaki Sugihara ◽  
Yukako Matsuki ◽  
Masafumi Yamane ◽  
...  

10.1038/83660 ◽  
2001 ◽  
Vol 27 (1) ◽  
pp. 103-107 ◽  
Author(s):  
Federica Di Palma ◽  
Ralph H. Holme ◽  
Elizabeth C. Bryda ◽  
Inna A. Belyantseva ◽  
Richard Pellegrino ◽  
...  

2021 ◽  
Vol 1 ◽  
pp. 100548
Author(s):  
A.D. Maier ◽  
A. Meddis ◽  
J. Haslund-Vinding ◽  
C. Mirian ◽  
A. Areskeviciute ◽  
...  

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi199-vi199
Author(s):  
Ramin Morshed ◽  
Alexander Haddad ◽  
Saket Jain ◽  
Sabraj Gill ◽  
Jordan Spatz ◽  
...  

Abstract Breast cancer is the most common malignancy in women in the United States, and brain metastases occur in almost a third of patients with metastatic dissemination. Immunoediting is a critical component of metastatic tumor cell elimination, and tumor clones that develop immune-escape mechanisms are associated with progression and metastatic dissemination. We hypothesized that breast cancer brain metastatic cells harbor immunomodulatory cytokine expression changes that promote an immunosuppressive environment to avoid immune cell-mediated elimination. To study this, a syngeneic mouse model of metastatic breast cancer was used. A brain metastatic line derived from the 4T1 breast cancer parental cell line was created by serially selecting brain metastatic populations of cells after intracardiac injection (4T1 BrM). A gene-expression analysis using an 800-gene cancer immunology-specific microarray panel was performed comparing the 4T1 parental and 4T1 BrM lines. 4T1 BrM cells demonstrate gene expression changes promoting immunosuppression including significant upregulation of IL18 and Lgals9 (Galectin-9) and downregulation of CD40, IL2rg, CCL2, and EOMES. When compared to 4T1 parental lines, the 4T1 BrM line demonstrated decreased expression of CCL2 and increased expression of GM-CSF on a cytokine array, corresponding to results obtained from gene expression analysis. These results suggest tumor-intrinsic cytokine expression changes that may mediate an immunosuppressive environment.


2017 ◽  
Vol 114 (36) ◽  
pp. 9695-9700 ◽  
Author(s):  
Alice Emptoz ◽  
Vincent Michel ◽  
Andrea Lelli ◽  
Omar Akil ◽  
Jacques Boutet de Monvel ◽  
...  

Our understanding of the mechanisms underlying inherited forms of inner ear deficits has considerably improved during the past 20 y, but we are still far from curative treatments. We investigated gene replacement as a strategy for restoring inner ear functions in a mouse model of Usher syndrome type 1G, characterized by congenital profound deafness and balance disorders. These mice lack the scaffold protein sans, which is involved both in the morphogenesis of the stereociliary bundle, the sensory antenna of inner ear hair cells, and in the mechanoelectrical transduction process. We show that a single delivery of the sans cDNA by the adenoassociated virus 8 to the inner ear of newborn mutant mice reestablishes the expression and targeting of the protein to the tips of stereocilia. The therapeutic gene restores the architecture and mechanosensitivity of stereociliary bundles, improves hearing thresholds, and durably rescues these mice from the balance defects. Our results open up new perspectives for efficient gene therapy of cochlear and vestibular disorders by showing that even severe dysmorphogenesis of stereociliary bundles can be corrected.


PLoS ONE ◽  
2012 ◽  
Vol 7 (2) ◽  
pp. e31490 ◽  
Author(s):  
Franck Thuny ◽  
Julien Textoris ◽  
Amira Ben Amara ◽  
Adil El Filali ◽  
Christian Capo ◽  
...  

2010 ◽  
Vol 16 (20) ◽  
pp. 5048-5057 ◽  
Author(s):  
Trent R. Hummel ◽  
Walter J. Jessen ◽  
Shyra J. Miller ◽  
Lan Kluwe ◽  
Victor F. Mautner ◽  
...  

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