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Conditional deletion of dnaic1 in a murine model of primary ciliary dyskinesia causes chronic rhinosinusitis.
Ostrowski LE, Yin W, Rogers TD, Busalacchi KB, Chua M, O'Neal WK, Grubb BR. Ostrowski LE, et al. Am J Respir Cell Mol Biol. 2010 Jul;43(1):55-63. doi: 10.1165/rcmb.2009-0118OC. Epub 2009 Aug 12. Am J Respir Cell Mol Biol. 2010. PMID: 19675306 Free PMC article.
To develop a viable mouse model for long-term studies of PCD, we have generated a transgenic mouse line in which two conserved exons of the mouse intermediate dynein chain gene, Dnaic1, are flanked by loxP sites (Dnaic1(flox/flox)). Dnaic1 is the murine homol …
To develop a viable mouse model for long-term studies of PCD, we have generated a transgenic mouse line in which two conserved exons of the …
Restoring ciliary function to differentiated primary ciliary dyskinesia cells with a lentiviral vector.
Ostrowski LE, Yin W, Patel M, Sechelski J, Rogers T, Burns K, Grubb BR, Olsen JC. Ostrowski LE, et al. Gene Ther. 2014 Mar;21(3):253-61. doi: 10.1038/gt.2013.79. Epub 2014 Jan 23. Gene Ther. 2014. PMID: 24451115 Free PMC article.
Mouse tracheal cells with a targeted deletion in the axonemal dynein intermediate chain 1 (Dnaic1) gene differentiate normally in culture but lack ciliary activity. Gene transfer to undifferentiated cultures of mouse Dnaic1(-/-) cells with a lentiviral vector pseudo …
Mouse tracheal cells with a targeted deletion in the axonemal dynein intermediate chain 1 (Dnaic1) gene differentiate normally in cul …
CRISPR/Cas9-Mediated Rapid Generation of Multiple Mouse Lines Identified Ccdc63 as Essential for Spermiogenesis.
Young SA, Miyata H, Satouh Y, Kato H, Nozawa K, Isotani A, Aitken RJ, Baker MA, Ikawa M. Young SA, et al. Int J Mol Sci. 2015 Oct 16;16(10):24732-50. doi: 10.3390/ijms161024732. Int J Mol Sci. 2015. PMID: 26501274 Free PMC article.
Using the advanced technology of CRISPR/Cas9 we have targeted three dynein group members; Dnaic1, Wdr63 and Ccdc63 in mice. All three of these genes are expressed strongly in the testis. We generated mice with amino acid substitutions in Dnaic1 to analyze two specif …
Using the advanced technology of CRISPR/Cas9 we have targeted three dynein group members; Dnaic1, Wdr63 and Ccdc63 in mice. All three …
Congenital heart disease and the specification of left-right asymmetry.
Francis RJ, Christopher A, Devine WA, Ostrowski L, Lo C. Francis RJ, et al. Am J Physiol Heart Circ Physiol. 2012 May 15;302(10):H2102-11. doi: 10.1152/ajpheart.01118.2011. Epub 2012 Mar 9. Am J Physiol Heart Circ Physiol. 2012. PMID: 22408017 Free PMC article.
To elucidate the role of left-right patterning in cardiovascular development, we examined situs anomalies and CHD in mice with a loss of function allele of Dnaic1, a dynein protein required for motile cilia function and left-right patterning. Dnaic1 mutants were fou …
To elucidate the role of left-right patterning in cardiovascular development, we examined situs anomalies and CHD in mice with a loss of fun …
Mucolytic treatment of chronic rhinosinusitis in a murine model of primary ciliary dyskinesia.
Yin W, Golliher HL, Ferguson AJ, Kimbell JS, Livraghi-Butrico A, Rogers TD, Grubb BR, Kimple AJ, Ostrowski LE. Yin W, et al. Front Mol Biosci. 2023 Jul 24;10:1221796. doi: 10.3389/fmolb.2023.1221796. eCollection 2023. Front Mol Biosci. 2023. PMID: 37555015 Free PMC article.
Objective: The goals of this study were to determine whether computed tomography (CT) imaging could be used to quantify mucus accumulation and whether the use of a mucolytic agent to reduce disulfide cross-links present in mucins would improve the effectiveness of nasal lavage at …
Objective: The goals of this study were to determine whether computed tomography (CT) imaging could be used to quantify mucus accumulation a …
Ion Torrent sequencing for conducting genome-wide scans for mutation mapping analysis.
Damerla RR, Chatterjee B, Li Y, Francis RJ, Fatakia SN, Lo CW. Damerla RR, et al. Mamm Genome. 2014 Apr;25(3-4):120-8. doi: 10.1007/s00335-013-9494-7. Epub 2013 Dec 5. Mamm Genome. 2014. PMID: 24306492 Free PMC article.
Further analysis of genes in the map interval identified a splicing mutation in Dnaic1(c.204+1G>A), an intermediate chain dynein, as the disease causing mutation in Bishu. ...
Further analysis of genes in the map interval identified a splicing mutation in Dnaic1(c.204+1G>A), an intermediate chain dynein, …
Mice with a Deletion of Rsph1 Exhibit a Low Level of Mucociliary Clearance and Develop a Primary Ciliary Dyskinesia Phenotype.
Yin W, Livraghi-Butrico A, Sears PR, Rogers TD, Burns KA, Grubb BR, Ostrowski LE. Yin W, et al. Am J Respir Cell Mol Biol. 2019 Sep;61(3):312-321. doi: 10.1165/rcmb.2017-0387OC. Am J Respir Cell Mol Biol. 2019. PMID: 30896965 Free PMC article.
Rsph1(-/-) animals accumulated mucus in the nasal cavity but had a lower bacterial burden than animals with a deletion of dynein axonemal intermediate chain 1 (Dnaic1(-/-)). Thus, Rsph1(-/-) mice display a PCD phenotype similar to but less severe than that observed in D
Rsph1(-/-) animals accumulated mucus in the nasal cavity but had a lower bacterial burden than animals with a deletion of dynein axonemal in …
Disruption in the autophagic process underlies the sensory neuropathy in dystonia musculorum mice.
Ferrier A, De Repentigny Y, Lynch-Godrei A, Gibeault S, Eid W, Kuo D, Zha X, Kothary R. Ferrier A, et al. Autophagy. 2015;11(7):1025-36. doi: 10.1080/15548627.2015.1052207. Autophagy. 2015. PMID: 26043942 Free PMC article.
LC3-II, SQTSM1, polyubiquitinated proteins and autophagic flux were also restored to wild-type levels in the rescued mice. Finally, a significant decrease in DNAIC1 (dynein, axonemal, intermediate chain 1; the mouse ortholog of human DNAI1), a member of the DMC (dynein/dyn …
LC3-II, SQTSM1, polyubiquitinated proteins and autophagic flux were also restored to wild-type levels in the rescued mice. Finally, a signif …