Key features and details
- Rabbit polyclonal to Dystrophin
- Suitable for: IHC-Fr, IHC-P
- Reacts with: Mouse, Human
- Isotype: IgG
- 倫理基準に準拠 - アニマル・フリーの生産
Dystrophin 一次抗体 製品一覧
製品の詳細Rabbit polyclonal to Dystrophin
アプリケーション適用あり: IHC-Fr, IHC-Pmore details
種交差性交差種: Mouse, Human
交差が予測される動物種: Rat, Dog, Pig
Synthetic peptide within Human Dystrophin aa 3650 to the C-terminus (C terminal). The exact sequence is proprietary.
Database link: P11532
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保存方法Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Preservative: 0.1% Sodium azide
Constituents: PBS, 1% BSA
Concentration information loading...
精製度Immunogen affinity purified
機能Anchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. Component of the dystrophin-associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. Also implicated in signaling events and synaptic transmission.
組織特異性Expressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Expressed in brain, muscle, kidney, lung and testis. Isoform 5 is expressed in heart, brain, liver, testis and hepatoma cells. Most tissues contain transcripts of multiple isoforms, however only isoform 5 is detected in heart and liver.
関連疾患Defects in DMD are the cause of Duchenne muscular dystrophy (DMD) [MIM:310200]. DMD is the most common form of muscular dystrophy; a sex-linked recessive disorder. It typically presents in boys aged 3 to 7 year as proximal muscle weakness causing waddling gait, toe-walking, lordosis, frequent falls, and difficulty in standing up and climbing up stairs. The pelvic girdle is affected first, then the shoulder girdle. Progression is steady and most patients are confined to a wheelchair by age of 10 or 12. Flexion contractures and scoliosis ultimately occur. About 50% of patients have a lower IQ than their genetic expectations would suggest. There is no treatment.
Defects in DMD are the cause of Becker muscular dystrophy (BMD) [MIM:300376]. BMD resembles DMD in hereditary and clinical features but is later in onset and more benign.
Defects in DMD are a cause of cardiomyopathy dilated X-linked type 3B (CMD3B) [MIM:302045]; also known as X-linked dilated cardiomyopathy (XLCM). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
配列類似性Contains 2 CH (calponin-homology) domains.
Contains 22 spectrin repeats.
Contains 1 WW domain.
Contains 1 ZZ-type zinc finger.
細胞内局在Cell membrane > sarcolemma. Cytoplasm > cytoskeleton.
- Information by UniProt
- BMD antibody
- CMD3B antibody
- DMD antibody
Muscle stem cells (from normal mouse) were injected into the gastric muscle of an MDX mouse. Dystrophin staining: primary antibody ab15277 and secondary antibody is donkey anti-rabbit Alexa 594.
This image was kindly supplied as part of the review submitted by Jessica Tebbets.
Immunofluorescence staining of dystrophin in W9, W987, and ESC. Myosin heavy chain (MHC) identified mouse muscle cells after differentiation. DAPI was used to stain nuclei.
Seventy-two hours before engraftment, 8 week-old mdx/SCID mice received 14 Gy of irradiation localized to the hind limb muscles. On the day of engraftment, SM/C-2.6-positive myogenic cells were purified by fluorescence-activated cell sorting (FACS), using a BD Aria II FACS machine and the same labeling protocol as described above for FC analysis, resuspended in 30 µl of phosphate buffered saline (PBS), loaded into an insulin syringe (BD), and injected into the left tibialis anterior (TA) muscle of anesthetized mice. 7.5×105 differentiated and sorted W987 cells were injected. Control mice were injected with PBS alone. Three weeks following engraftment, TA muscles were harvested, fixed in 0.5% paraformaldehyde for 4 hours, dehydrated in 20% sucrose overnight and frozen in optimal cutting temperature (OCT) using liquid nitrogen cooled methyl-butane. Tissue blocks imbedded in OCT were cryosectioned and processed for immunocytochemical analysis using rabbit anti-dystrophin. Secondary antibodies used were donkey anti-rabbit conjugated to Alexafluor 594 and donkey anti-rat conjugated to Alexafluor 488 (Life Technologies). Nuclei were visualized using NucBlue Fixed Cell Stain (Life Technologies).
Gene-corrected mdx iPSC W987, non-gene-corrected unexcised mdx iPSC W9 and wild-type ESC controls.
Dystrophin quantification in a population of myofibres identified in entire muscle sections performing the double labelling anti-dystrophin ab15277 (red; 1/200 dilution) and anti-spectrin (green; 1/20 dilution).
All the labellings were performed at RT. Human Muscle sections were incubated with the primary antibody combination (anti-dystrophin ab15277 and anti-spectrin) for 1 hour. After three washes with PBS sections were incubated with Alexa Fluor 488 conjugated anti-mouse IgG (1:100, Thermo Fisher Scientific, Hemel Hempstead, UK) and anti-rabbit biotinylated IgG (1:200; GE Healthcare, Amersham Pl, UK) for 30 minutes. PBS washes were performed and sections were incubated with Alexa Fluor 594 streptavidin conjugate (1:1000, Thermo Fisher Scientific, Hemel Hempstead, UK).
Representative images of entire muscle sections stained and acquired by the Axio Scan slide scanner and processed with Definens algorithm derived from a control (a) and from a DMD patient (b).
DMD: Duchenne Muscular Dystrophy.
Immunohistochemical staining of human skeletal muscle with ab15277
ab15277 は 317 報の論文で使用されています。
- Tone Y et al. Immortalized Canine Dystrophic Myoblast Cell Lines for Development of Peptide-Conjugated Splice-Switching Oligonucleotides. Nucleic Acid Ther 31:172-181 (2021). PubMed: 33567244
- Xuan W et al. Pluripotent stem cell-induced skeletal muscle progenitor cells with givinostat promote myoangiogenesis and restore dystrophin in injured Duchenne dystrophic muscle. Stem Cell Res Ther 12:131 (2021). PubMed: 33579366
- Simmons TR et al. Pre-clinical dose-escalation studies establish a therapeutic range for U7snRNA-mediated DMD exon 2 skipping. Mol Ther Methods Clin Dev 21:325-340 (2021). PubMed: 33898631
- Xiang X et al. Efficient correction of Duchenne muscular dystrophy mutations by SpCas9 and dual gRNAs. Mol Ther Nucleic Acids 24:403-415 (2021). PubMed: 33868784
- Yao X et al. Engineered extracellular vesicles as versatile ribonucleoprotein delivery vehicles for efficient and safe CRISPR genome editing. J Extracell Vesicles 10:e12076 (2021). PubMed: 33747370