货号：ABN61

品牌：Merck Millipore

规格：

目录价：￥4383.00

市场价格：￥3725.55

会员价格：￥3506.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Alpha-dystrobrevin Antibody | ABN61 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Molecular Weight: ~45/80 kDa observed. Uniprot describes 8 isoforms produced by alternative splicing ranging from 22-84 kDa. Uncharacterized bands may appear at ~18 and ~57 kDa in some lysates. View All» Immunogen: GST-tagged recombinant protein corresponding to human Alpha-dystrobrevin. View All» Background Information: 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. View All» Species Reactivity:

• Human

• Mouse
  View All» Species Reactivity Note: Demonstrated to react with Human and Mouse. No homology to Rat was found. View All» Control: Human brain tissue lysate View All» Quality Assurance: 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. View All» Purification Method: Affinity purified View All» Presentation: Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: Q9Y4J8 View All» Entrez Gene Number: NP_001381 View All» Gene Symbol:
  • DTNA

  • DRP3
    View All» Alternate Names:
    • Dystrobrevin alpha

    • DTN-A

    • Alpha-dystrobrevin

    • Dystrophin-related protein 3
      View All» Usage Statement: 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. View All» Key Applications: Western Blotting View All» Entrez Gene Summary: 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]. View All» UniProt Summary: 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. View All» Product Name: Anti-Alpha-dystrobrevin View All» Concentration: 1.0 mg/mL View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Affinity Purified View All» Host: Rabbit View All»
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