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    Desmin / PerCP (bs-1026R-PerCP)  
    訂購熱線:400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
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    100ul/2980.00元
    大包裝/詢價
    產品編號 bs-1026R-PerCP
    英文名稱 Desmin / PerCP
    中文名稱 PerCP標記的結蛋白抗體
    別    名 DESM_HUMAN; DES;  
    研究領域 腫瘤  心血管  免疫學  信號轉導  細胞類型標志物  
    抗體來源 Rabbit
    克隆類型 Polyclonal
    交叉反應
    產品應用
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 52kDa
    細胞定位 細胞漿 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human Desmin: 261-360/470 
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    保存條件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
    注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    PubMed PubMed
    產品介紹 Desmin is a muscle-specific, type III intermediate filament that integrates the sarcolemma, Z disk, and nuclear membrane in sarcomeres and regulates sarcomere architecture. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z line structures. Defects in Desmin are the cause of desmin related cardio skeletal myopathy (CSM) also known as desmin related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin reactive deposits in cardiac and skeletal muscle cells. A desmin related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM). Defects in Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. Antidesmin antibodies are useful in identification of tumours of myogenic origin.

    SWISS:
    P17661

    Gene ID:
    1674

    Database links:

    Entrez Gene: 1674 Human

    Entrez Gene: 13346 Mouse

    Entrez Gene: 64362 Rat

    Omim: 125660 Human

    SwissProt: P17661 Human

    SwissProt: P31001 Mouse

    SwissProt: P48675 Rat

    Unigene: 594952 Human

    Unigene: 6712 Mouse

    Unigene: 39196 Rat



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