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    CCDC39 Rabbit pAb (bs-8096R)  
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    產(chǎn)品編號 bs-8096R
    英文名稱(chēng) CCDC39 Rabbit pAb
    中文名稱(chēng) 卷曲螺旋結構域蛋白39抗體
    別    名 CCD39_HUMAN; Ccdc39; Coiled-coil domain-containing protein 39.  
    研究領(lǐng)域 細胞生物  免疫學(xué)  信號轉導  細胞骨架  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 Human (predicted: Mouse,Rat,Rabbit,Cow,Horse)
    產(chǎn)品應用 WB=1:500-2000
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 110 kDa
    檢測分子量
    細胞定位 細胞漿 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human CCDC39: 651-750/941 
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    保存條件 Shipped at 4℃. Store at -20℃ 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(chǎn)品介紹 Required for assembly of dynein regulatory complex (DRC) and inner dynein arm complexes, which are responsible for ciliary beat regulation, thereby playing a central role in motility in cilia and flagella. Not required for outer dynein arm complexes assembly.
    Tissue specificity:Mainly expressed in nasal brushings and, to a lesser extent, in lungs and testis.
    Involvement in disease:
    Defects in CCDC39 are the cause of primary ciliary dyskinesia type 14 (CILD14) . A disorder characterized by abnormalities of motile cilia. Respiratory infections leading to chronic inflammation and bronchiectasis are recurrent, due to defects in the respiratory cilia; reduced fertility is often observed in male patients due to abnormalities of sperm tails. Half of the patients exhibit randomization of left-right body asymmetry and situs inversus, due to dysfunction of monocilia at the embryonic node. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome.

    Function:
    Required for assembly of dynein regulatory complex (DRC) and inner dynein arm complexes, which are responsible for ciliary beat regulation, thereby playing a central role in motility in cilia and flagella. Not required for outer dynein arm complexes assembly.

    Subcellular Location:
    Cytoplasm, cytoskeleton, cilium axoneme. Note=CCDC40 is required for localization to axonemes.

    Tissue Specificity:
    Mainly expressed in nasal brushings and, to a lesser extent, in lungs and testis.

    DISEASE:
    Defects in CCDC39 are the cause of primary ciliary dyskinesia type 14 (CILD14) [MIM:613807]. A disorder characterized by abnormalities of motile cilia. Respiratory infections leading to chronic inflammation and bronchiectasis are recurrent, due to defects in the respiratory cilia; reduced fertility is often observed in male patients due to abnormalities of sperm tails. Half of the patients exhibit randomization of left-right body asymmetry and situs inversus, due to dysfunction of monocilia at the embryonic node. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome.

    Similarity:
    Belongs to the CCDC39 family.

    SWISS:
    Q9UFE4

    Gene ID:
    339829

    Database links:

    Entrez Gene: 488089 Dog

    Entrez Gene: 339829 Human

    Entrez Gene: 51938 Mouse

    Omim: 613798 Human

    SwissProt: E2R1I5 Dog

    SwissProt: Q9UFE4 Human

    SwissProt: Q9D5Y1 Mouse

    Unigene: 712820 Human

    Unigene: 474546 Mouse



    產(chǎn)品圖片
    Sample: DU145(Human) Cell Lysate at 30 ug Primary: Anti- CCDC39 (bs-8096R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 110 kD Observed band size: 110 kD
    Sample: HL-60(Human) Cell Lysate at 30 ug Primary: Anti- CCDC39 (bs-8096R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 110 kD Observed band size: 110 kD
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