2002
DOI: 10.1089/10430340260185085
|View full text |Cite
|
Sign up to set email alerts
|

Abstract: Duchenne muscular dystrophy (DMD) is the most common disabling and lethal genetic muscle disorder, afflicting 1 of every 3500 males. Patients with DMD experience progressive muscle degeneration and weakness and succumb to respiratory or cardiac failure by their early twenties. No treatment is currently available for DMD. Mutations in the dystrophin gene result in lack of a functional dystrophin protein in striated muscle, which induces instability in the muscle cell membrane leading to persistent muscle injury… Show more

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...
2
1
1
1

Citation Types

2
95
0
3

Year Published

2004
2004
2017
2017

Publication Types

Select...
7

Relationship

2
5

Authors

Journals

citations
Cited by 120 publications
(100 citation statements)
references
References 57 publications
2
95
0
3
Order By: Relevance
“…In our previous studies, we abbreviated the essential functional units of the human dystrophin gene down to less than 4.2 kb and showed that these minidystrophin vectors successfully ameliorated the dystrophic phenotypes. 4,5 As depicted in Figure 1, in this study, we followed the same rationale and constructed a truncated canine minidystrophin gene (3.8 kb) consisted of the following elements: N terminus, hinge1, rods 1, 2, 22, 23, 24, hinge 4 and CR domain (Figure 1a). This canine minigene was under the transcriptional control by a human cytomegalovirus immediate-early gene (CMV) promoter for strong and long-term gene expression in the muscle.…”
Section: Cloning and Construction Of The Aav1-cminidys Vectormentioning
confidence: 99%
See 2 more Smart Citations
“…In our previous studies, we abbreviated the essential functional units of the human dystrophin gene down to less than 4.2 kb and showed that these minidystrophin vectors successfully ameliorated the dystrophic phenotypes. 4,5 As depicted in Figure 1, in this study, we followed the same rationale and constructed a truncated canine minidystrophin gene (3.8 kb) consisted of the following elements: N terminus, hinge1, rods 1, 2, 22, 23, 24, hinge 4 and CR domain (Figure 1a). This canine minigene was under the transcriptional control by a human cytomegalovirus immediate-early gene (CMV) promoter for strong and long-term gene expression in the muscle.…”
Section: Cloning and Construction Of The Aav1-cminidys Vectormentioning
confidence: 99%
“…Twenty-four hours after transfection, the cells were harvested and lysed in 100 ml of radioimmunoprecipitation assay buffer (10 mM Tris-HCl pH8.2, 1% Triton X-100, 1% SDS and 150 mM NaCl). The western blot analysis was performed as described before 5 utilizing polyclonal antibody directed against the rods 1 and 2 region of human dystrophin, which has cross-reaction with canine dystrophin (1:5000).…”
Section: Western Analysis For the Evaluation Of Canine Minidystrophinmentioning
confidence: 99%
See 1 more Smart Citation
“…The medium was changed twice with Dulbecco's modified Eagle's medium containing 10% fetal bovine serum and antibiotics (1% Pen/Strep) at 6 and 48 h after transfection. The activation of NF-kB signaling pathway was activated by stimulation with lipopolysaccharide (Sigma) for 15 min, and cells were harvested for western blot assay that was carried out, according to a previously published method, 18 to determine the levels of P-p65 in mouse macrophages following the lipopolysaccharide stimulation. Cells were lysed in 20 ml of radioimmunoprecipitation assay buffer.…”
Section: Western Blot Analysismentioning
confidence: 99%
“…Cells were lysed in 20 ml of radioimmunoprecipitation assay buffer. 18 After adding loading buffer, all samples were boiled for 5 min and then were chilled on ice for 10 min. The samples were electrophoretically separated on 8% SDSpolyacrylamide gel, transferred to an Immobilon-P Transfer Membrane (Millipore, Tullagreen, Carrigtohill, Ireland), and analyzed by western blotting with phospho-NF-kB/65 (Ser536) (93H1) rabbit mAb (Cell Signaling) as the primary antibody and a goat anti-rabbit IgG (H þ L) as the secondary antibody (Thermo Fisher Scientific, Peroxidase Conjugated, 1:5000, Rockford, IL USA).…”
Section: Western Blot Analysismentioning
confidence: 99%