Key features and details
- Mouse monoclonal [MANDRA1] to Dystrophin
- Suitable for: IHC-Fr
- Reacts with: Human
- Isotype: IgG1
参阅全部 Dystrophin 一抗
描述小鼠单克隆抗体[MANDRA1] to Dystrophin
经测试应用适用于: IHC-Frmore details
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.
表位128 amino acids at the end of the C-terminal domain of the human dystrophin molecule (a.a. residues 3558-3684).
- lympho blastoid cells, cultures of brain astroglial and neuronal cells, liver and Hep G2 cells
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.
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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
Concentration information loading...
纯度Tissue culture supernatant
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).
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Use at an assay dependent concentration.
Use at an assay dependent concentration.
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
- BMD antibody
- CMD3B antibody
- DMD antibody
ab7164 被引用在 18 文献中.
- Olie CS et al. Cytoskeletal disorganization underlies PABPN1-mediated myogenic disability. Sci Rep 10:17621 (2020). PubMed: 33077830
- Uezu A et al. Essential role for InSyn1 in dystroglycan complex integrity and cognitive behaviors in mice. Elife 8:N/A (2019). PubMed: 31829939
- Baruffaldi F et al. Dynamic Phosphorylation of the Myocyte Enhancer Factor 2Ca1 Splice Variant Promotes Skeletal Muscle Regeneration and Hypertrophy. Stem Cells 35:725-738 (2017). PubMed: 27612437
- Sekulic-Jablanovic M et al. Functional characterization of orbicularis oculi and extraocular muscles. J Gen Physiol 147:395-406 (2016). PubMed: 27069119
- Karolczak J et al. Myosin VI in skeletal muscle: its localization in the sarcoplasmic reticulum, neuromuscular junction and muscle nuclei. Histochem Cell Biol 139:873-85 (2013). PubMed: 23275125