货号：MAB1692

品牌：Merck Millipore

规格：1mL

目录价：￥4975.00

市场价格：￥4228.75

会员价格：￥3980.00

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Dystrophin Antibody, mid-rod, clone 6D3 | MAB1692 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Mid rod domain (between amino acids 1181 and 1388) of human dystrophin. Also reacts with skeletal, cardiac and smooth muscle dystrophin from normal mouse, rat, rabbit and dog. Other animal species have not been tested. Reacts on blots with the brain isoform. No reactivity with mdx mouse tissue or DMD/BMD patients who have a gene deletion which removes the antibody binding site. Does not react with chicken dystrophin. STAINING PATTERN:Light microscopy: continuous rim of labeling at the periphery of muscle fibers. E.M. gold: close to the cytoplasmic face of the plasma membrane. Western blotting: strong double bands at approximately 400 kD plus metabolites of lower molecular mass. View All» Epitope: mid-rod View All» Immunogen: Bacterial fusion protein (Cell (1987) 51:919-928). View All» Clone: 6D3 View All» Isotype: IgG2a View All» Species Reactivity:

• Canine

• Human

• Mouse

• Rat

• Rabbit
  View All» Application Notes: Immunohistochemistry: (fresh frozen, unfixed tissue only): use undiluted - 1:20. Not recommended for use on paraffin embedded tissue. EM Gold (Light fixation with 2% formaldehyde + 0.001% glutaraldehyde for 1 hour. 2.3M sucrose used as cryoprotectant.): use undiluted. 90 minute incubation at 25°C. Western blotting: use 1:100-1:250. Optimal working dilutions must be determined by the end user. Protocol for Immunohistochemical use of MAB1692 1) Freeze muscle blocks in isopentane chilled in liquid nitrogen. 2) Cut 4 μm to 10 μm sections and air dry on slides coated with 0.5% gelatin containing 0.05% chrome alum. 3) Slides may be stored at -70 °C wrapped in cling film until required. If stored sections are used, allow sections to equilibrate to room temperature before unwrapping and proceeding. 4) Apply a 50 μL aliquot of primary antibody to sections (unfixed). Incubate for 1 hour at room temperature or 37°C. 5) Wash sections 3 x 10 minutes in phosphate buffered saline. 6) Apply a 50 μL aliquot of labeled second antibody. Incubate for 60 minutes at 25°C. 7) Wash sections 3 x 10 minutes in phosphate buffered saline. 8) Mount fluorescent sections in aqueous mounting media or visualize peroxidase label (DAB). Dehydrate, clean and mount peroxidase labeled sections for permanent preparations. View All» Control: POSITIVE CONTROL: Snap frozen normal human or rat striated muscle. View All» Presentation: Culture supernatant, liquid in PBS with 1% BSA, containing 15 mM sodium azide. View All» Storage Conditions: Maintain at -20°C for up to one year in convenient undiluted aliquots. Avoid repeated freeze/thaw cycles. View All» UniProt Number: P11532 View All» Entrez Gene Number:
  • NM_000109.2

  • NM_004006.1

  • NM_004007.1

  • NM_004009.2

  • NM_004010.2

  • NM_004011.2

  • NM_004012.2

  • NM_004013.1

  • NM_004014.1

  • NM_004015.1

  • NM_004016.1

  • NM_004017.1

  • NM_004018.1

  • NM_004019.2

  • NM_004020.1

  • NM_004021.1

  • NM_004022.1

  • NM_004023.1
    View All» Gene Symbol:
    • DMD

    • DXS272

    • DXS269

    • DXS230

    • Dystrophin.

    • DXS268

    • DXS270

    • DXS239

    • DXS142

    • CMD3B

    • DXS206

    • dystrophin

    • DXS164

    • BMD
      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

      • Immunohistochemistry
        View All» Entrez Gene Summary: The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. View All» UniProt Summary: FUNCTION: SwissProt: P11532 # May play a role in anchoring the cytoskeleton to the plasma membrane.SIZE: 3685 amino acids; 426692 Da SUBUNIT: Interacts with the syntrophins SNTA1, SNTB1, SNTB2, SNTG1 and SNTG2. Interacts with KRT19.SUBCELLULAR LOCATION: Cell membrane, sarcolemma; Peripheral membrane protein; Cytoplasmic side. Cytoplasm, cytoskeleton.TISSUE SPECIFICITY: Expressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Isoform 5 is expressed in brain, liver, testis and hepatoma cells.DISEASE: SwissProt: P11532 # 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 dilated cardiomyopathy [MIM:302045]; also known as X-linked dilated cardiomyopathy (XLCM). Dystrophin mutations may predispose to common sporadic cardiomyopathy cases.SIMILARITY: SwissProt: P11532 ## Contains 2 CH (calponin-homology) domains. & Contains 22 spectrin repeats. & Contains 1 WW domain. & Contains 1 ZZ-type zinc finger.MISCELLANEOUS: The DMD gene is the largest known gene in humans. It is 2.4 million base-pairs in size, comprises 79 exons and takes over 16 hours to be transcribed and cotranscriptionally spliced. View All» Brand Family: Chemicon View All» Product Name: Anti-Dystrophin Antibody, mid-rod, clone 6D3 | MAB1692 View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 1 mL View All» Format: Culture Supernatant View All» Host: Mouse View All»
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