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    Rabbit Anti-KBTBD13/Gold Conjugated antibody (bs-17086R-Gold)
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    說(shuō) 明 書(shū): 100ul(10nm  15nm  35nm
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-17086R-Gold
    英文名稱(chēng) Rabbit Anti-KBTBD13/Gold Conjugated antibody
    中文名稱(chēng) 膠體金標記的KBTBD13蛋白抗體
    別    名 5430433E21Rik; HCG1645727; KBTBD_HUMAN; Kbtbd13; Kelch repeat and BTB (POZ) domain containing 13; Kelch repeat and BTB domain-containing protein 13; NEM6.  
    規格價(jià)格 100ul/2980元 購買(mǎi)        大包裝/詢(xún)價(jià)
    說(shuō) 明 書(shū) 100ul(10nm  15nm  35nm
    研究領(lǐng)域 細胞生物  免疫學(xué)  發(fā)育生物學(xué)  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 (predicted: Human, Mouse, Cow, )
    產(chǎn)品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 49kDa
    性    狀 Lyophilized or Liquid
    濃    度 0.4mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human KBTBD13
    亞    型 IgG
    純化方法 affinity purified by Protein A
    儲 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
    保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
    產(chǎn)品介紹 background:
    The gene belongs to a family of genes encoding proteins containing a BTB domain and several kelch repeats. The BTB domain functions as a protein-protein interaction module, which includes an ability to self-associate or to interact with non-BTB domain-containing proteins. The kelch motif typically occurs in groups of five to seven repeats, and has been found in proteins with diverse functions. Known functions of these family members include transcription regulation, ion channel tetramerization and gating, protein ubiquitination or degradation, and cytoskeleton regulation. The exact function of this family member has yet to be determined. [provided by RefSeq, Jun 2010]

    Subcellular Location:
    Cytoplasm.

    Tissue Specificity:
    Expressed in skeletal muscle.

    DISEASE:
    Defects in KBTBD13 are the cause of nemaline myopathy type 6 (NEM6) [MIM:609273]. A form of nemaline myopathy characterized by childhood onset of slowly progressive proximal muscle weakness, exercise intolerance, and slow movements with stiff muscles. Patients are unable to run or correct themselves from falling over.

    Similarity:
    Contains 1 BTB (POZ) domain.
    Contains 5 Kelch repeats.

    Database links:

    Entrez Gene: 390594 Human

    Omim: 613727 Human

    SwissProt: C9JR72 Human

    Unigene: 586890 Human



    Important Note:
    This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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