Anti-Dystrophin 抗体 (ab15277)
Key features and details
- Rabbit polyclonal to Dystrophin
- Suitable for: IHC-Fr, IHC-P
- Reacts with: Mouse, Human
- Isotype: IgG
リコンビナント抗体で、ロット間での高い再現性を実現
- 異なるロット間での安定した再現性
- 容易なスケールアップ
- 評価試験による特異性の確認済み
- 倫理基準に準拠 - アニマル・フリーの生産
製品の概要
-
製品名
Anti-Dystrophin antibody
Dystrophin 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to Dystrophin -
由来種
Rabbit -
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 -
免疫原
Synthetic peptide within Human Dystrophin aa 3650 to the C-terminus (C terminal). The exact sequence is proprietary.
Database link: P11532 -
特記事項
This product is FOR RESEARCH USE ONLY. For commercial use, please contact partnerships@abcam.com.
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
製品の特性
-
製品の状態
Liquid -
保存方法
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. -
バッファー
pH: 7.60
Preservative: 0.1% Sodium azide
Constituents: PBS, 1% BSA -
Concentration information loading...
-
精製度
Immunogen affinity purified -
ポリ/モノ
ポリクローナル -
アイソタイプ
IgG -
研究分野
関連製品
-
Compatible Secondaries
-
Isotype control
-
Recombinant Protein
アプリケーション
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab15277の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
アプリケーション | Abreviews | 特記事項 |
---|---|---|
IHC-Fr | (6) |
1/400. Use with acetone-fixed tissues.
|
IHC-P | (14) |
1/100.
|
特記事項 |
---|
IHC-Fr
1/400. Use with acetone-fixed tissues. |
IHC-P
1/100. |
ターゲット情報
-
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
-
参照データベース
- Entrez Gene: 1756 Human
- Entrez Gene: 13405 Mouse
- Entrez Gene: 24907 Rat
- NCBI: NP_004002.2 Human
- Omim: 300377 Human
- SwissProt: O97592 Dog
- SwissProt: P11532 Human
- SwissProt: P11531 Mouse
see all -
別名
- BMD antibody
- CMD3B antibody
- DMD antibody
see all
画像
-
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
データシートおよび資料
-
SDS download
-
Datasheet download
参考文献 (426)
ab15277 は 426 報の論文で使用されています。
- Nolt GL et al. Inhibition of p53-MDM2 binding reduces senescent cell abundance and improves the adaptive responses of skeletal muscle from aged mice. Geroscience 46:2153-2176 (2024). PubMed: 37872294
- Jollet M et al. Insight into the Role of Gut Microbiota in Duchenne Muscular Dystrophy: An Age-Related Study in Mdx Mice. Am J Pathol 194:264-279 (2024). PubMed: 37981219
- Bittel AJ et al. Voluntary wheel running improves molecular and functional deficits in a murine model of facioscapulohumeral muscular dystrophy. iScience 27:108632 (2024). PubMed: 38188524
- Shiba N et al. Efficacy of exon-skipping therapy for DMD cardiomyopathy with mutations in actin binding domain 1. Mol Ther Nucleic Acids 34:102060 (2023). PubMed: 38028197
- Esper ME et al. Immunofluorescence Labeling of Skeletal Muscle in Development, Regeneration, and Disease. Methods Mol Biol 2566:113-132 (2023). PubMed: 36152246