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品种反应性 主要应用 宿主 格式 抗体类型 M, H WB Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 ABN61 描述 Anti-Alpha-dystrobrevin Antibody Alternate Names - Dystrophin-related protein 3
- Dystrobrevin alpha
- DTN-A
- Alpha-dystrobrevin
背景信息 Alpha-dystrobrevin belongs to the dystropohin family and is a cytoplasmic component of the dystrophin-associated protein complex. The complex localizes to the sarcolemma and its disruption is associated with various forms of muscular dystrophy. Defects in Alpha-dystrobrevin arrests myocardial morphogenesis leading to left ventricular non-compaction type 1. This disorder is characterized by a hypertrophic left ventricule with deep trabeculations and with poor systolic function, with or without associated left ventricular dilation. In some cases, it is associated with other congenital heart anomalies such as ventricular septal defects, pulmonic stenosis and atrial septal defects. The right ventricle may also be affected. 产品信息 格式 Affinity Purified 控制 - Human brain tissue lysate
演示 Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. 应用 应用 Anti-Alpha-dystrobrevin Antibody detects level of Alpha-dystrobrevin & has been published & validated for use in WB. 主要应用 - Western Blotting
生物信息 免疫原品种 GST-tagged recombinant protein corresponding to human Alpha-dystrobrevin. 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rabbit 品种反应性 MouseHuman Species Reactivity Note Demonstrated to react with Human and Mouse. No homology to Rat was found. 抗体类型 Polyclonal Antibody Entrez基因编号 - NP_001381
Entrez基因汇总 The protein encoded by this gene belongs to the dystrobrevin subfamily of the dystrophin family. This protein is a component of the dystrophin-associated protein complex (DPC), which consists of dystrophin and several integral and peripheral membrane proteins, including dystroglycans, sarcoglycans, syntrophins and alpha- and beta-dystrobrevin. The DPC localizes to the sarcolemma and its disruption is associated with various forms of muscular dystrophy. Mutations in this gene are associated with left ventricular noncompaction with congenital heart defects. Multiple alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jul 2008]. 基因符号 - DRP3
- DTNA
纯化方法 Affinity purified UniProt编号 - Q9Y4J8
UniProt汇总 FUNCTION: May be involved in the formation and stability of synapses as well as being involved in the clustering of nicotinic acetylcholine receptors.
SUBUNIT STRUCTURE: Interacts with dystrophin, utrophin and the syntrophins SNTA1, SNTB1, SNTB2, SNTG1 and SNTG2. Isoform 7 and isoform 8 do not interact with dystrophin. Binds dystrobrevin binding protein 1. Interacts with MAGEE1 (By similarity).
SUBCELLULAR LOCATION: Cytoplasm. Cell junction › synapse. Cell membrane (By similarity). Note: In peripheral nerves, co-localizes with MAGEE1 in the Schwann cell membrane (By similarity).
TISSUE SPECIFICITY: Highly expressed in brain, skeletal and cardiac muscles, and expressed at lower levels in lung, liver and pancreas. Isoform 2 is not expressed in cardiac muscle. Isoform 7 and isoform 8 are only expressed in muscle.
DOMAIN: The coiled coil domain mediates the interaction with dystrophin and utrophin (By similarity).
PTM: Phosphorylation of DTN-1 on tyrosine kinase substrate domain present in the C-terminus (By similarity).
INVOLVEMENT IN DISEASE: Defects in DTNA are the cause of left ventricular non-compaction type 1 (LVNC1) [MIM:604169]. Left ventricular non-compaction is due to an arrest of myocardial morphogenesis. The disorder is characterized by a hypertrophic left ventricule with deep trabeculations and with poor systolic function, with or without associated left ventricular dilation. In some cases, it is associated with other congenital heart anomalies such as ventricular septal defects, pulmonic stenosis and atrial septal defects. The right ventricle may also be affected.
SEQUENCE SIMILARITIES: Belongs to the dystrophin family. Dystrobrevin subfamily.
Contains 1 ZZ-type zinc finger.产品使用声明 质量保证 Evaluated by Western Blot in human brain tissue lysate.
Western Blot Analysis: 1 µg/mL of this antibody detected Alpha-dystrobrevin on 10 µg of human brain tissue lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
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