Key features and details
- Mouse monoclonal [MANDRA1] to Dystrophin
- Suitable for: IHC-Fr
- Reacts with: Mouse, Rat, Human, Fish
- Isotype: IgG1
製品名Anti-Dystrophin antibody [MANDRA1]
Dystrophin 一次抗体 製品一覧
製品の詳細Mouse monoclonal [MANDRA1] to Dystrophin
アプリケーション適用あり: IHC-Frmore details
種交差性交差種: Mouse, Rat, Human, Fish
Fusion protein, corresponding to amino acids 3200-3684 of Human Dystrophin.
エピトープ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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保存方法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.
バッファーPreservative: 0.097% Sodium azide
Concentration information loading...
精製度Tissue culture supernatant
一次抗体 備考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).
Our Abpromise guarantee covers the use of ab7164 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-Fr||Use at an assay dependent concentration.|
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
ab7164 は 17 報の論文で使用されています。
- 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
- Wei-Lapierre L et al. Orai1-dependent calcium entry promotes skeletal muscle growth and limits fatigue. Nat Commun 4:2805 (2013). IHC ; Mouse . PubMed: 24241282