Anti-Dystrophin抗体[MANDRA1] (ab7164)
Key features and details
- Mouse monoclonal [MANDRA1] to Dystrophin
- Suitable for: IHC-Fr, WB, ELISA, ICC/IF
- Reacts with: Mouse, Rat, Human, Fish
- Isotype: IgG1
概述
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产品名称
Anti-Dystrophin抗体[MANDRA1]
参阅全部 Dystrophin 一抗 -
描述
小鼠单克隆抗体[MANDRA1] to Dystrophin -
宿主
Mouse -
经测试应用
适用于: IHC-Fr, WB, ELISA, ICC/IFmore details
不适用于: IHC-P -
种属反应性
与反应: Mouse, Rat, Human, Fish -
免疫原
Recombinant fragment within Human Dystrophin aa 3200-3700. The exact immunogen sequence used to generate this antibody is proprietary information. If additional detail on the immunogen is needed to determine the suitability of the antibody for your needs, please contact our Scientific Support team to discuss your requirements.
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表位
128 amino acids at the end of the C-terminal domain of the human dystrophin molecule (a.a. residues 3558-3684). -
阳性对照
- lympho blastoid cells, cultures of brain astroglial and neuronal cells, liver and Hep G2 cells
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常规说明
The C-terminal domain of the human dystrophin molecule (a.a. residues 3558-3684) is present in normal muscle tissue. It is also present in nearly all Becker muscular dystrophies, but is absent in cases of Duchenne muscular dystrophies and in the dystrophic mouse (mdx).
This product was changed from ascites to tissue culture supernatant on 17 May 2019. Please note that the dilutions may need to be adjusted accordingly. If you have any questions, please do not hesitate to contact our scientific support team.
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
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle. -
存储溶液
Preservative: 0.097% Sodium azide
Constituent: 0.411% PBS -
Concentration information loading...
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纯度
Proprietary Purification -
纯化说明
Purified from culture supernatant of hybridoma cells with proprietary method. -
Primary antibody说明
The C-terminal domain of the human dystrophin molecule (a.a. residues 3558-3684) is present in normal muscle tissue. It is also present in nearly all Becker muscular dystrophies, but is absent in cases of Duchenne muscular dystrophies and in the dystrophic mouse (mdx). -
克隆
单克隆 -
克隆编号
MANDRA1 -
同种型
IgG1 -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
应用
应用 | Ab评论 | 说明 |
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IHC-Fr |
Use at an assay dependent concentration.
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WB | (1) |
Use at an assay dependent concentration.
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ELISA |
Use at an assay dependent concentration.
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ICC/IF |
Use at an assay dependent concentration. PubMed: 22869749
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说明 |
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IHC-Fr
Use at an assay dependent concentration. |
WB
Use at an assay dependent concentration. |
ELISA
Use at an assay dependent concentration. |
ICC/IF
Use at an assay dependent concentration. PubMed: 22869749 |
靶标
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功能
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. -
组织特异性
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. -
疾病相关
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. -
序列相似性
Contains 2 CH (calponin-homology) domains.
Contains 22 spectrin repeats.
Contains 1 WW domain.
Contains 1 ZZ-type zinc finger. -
细胞定位
Cell membrane > sarcolemma. Cytoplasm > cytoskeleton. - Information by UniProt
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数据库链接
- Entrez Gene: 1756 Human
- Entrez Gene: 13405 Mouse
- Entrez Gene: 24907 Rat
- NCBI: NP_004002.2 Human
- Omim: 300377 Human
- SwissProt: P11532 Human
- SwissProt: P11531 Mouse
- SwissProt: P11530 Rat
see all -
别名
- BMD antibody
- CMD3B antibody
- DMD antibody
see all
图片
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ab7164 staining Dystrophin in frozen human tongue tissue sections by Immunohistochemistry (IHC - Fr- Frozen sections). Samples were incubated 1:100 dilution. A Goat Anti-mouse, FITC- conjugate was used as the secondary antibody.
This image was generated using the ascites version of the product.
实验方案
数据表及文件
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SDS download
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Datasheet download
文献 (23)
ab7164 被引用在 23 文献中.
- Lange J et al. Dystrophin deficiency affects human astrocyte properties and response to damage. Glia 70:466-490 (2022). PubMed: 34773297
- Wang R et al. Resveratrol ameliorates muscle atrophy in chronic kidney disease via the axis of SIRT1/FoxO1. Phytother Res 36:3265-3275 (2022). PubMed: 35606908
- Rugerio-Martínez CI et al. Dp71 Point Mutations Induce Protein Aggregation, Loss of Nuclear Lamina Integrity and Impaired Braf35 and Ibraf Function in Neuronal Cells. Int J Mol Sci 23:N/A (2022). PubMed: 36233175
- Wang T et al. Anti-VEGF therapy prevents Müller intracellular edema by decreasing VEGF-A in diabetic retinopathy. Eye Vis (Lond) 8:13 (2021). PubMed: 33865457
- Olie CS et al. Cytoskeletal disorganization underlies PABPN1-mediated myogenic disability. Sci Rep 10:17621 (2020). PubMed: 33077830