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    Rabbit Anti-LRRC6/AP Conjugated antibody (bs-13613R-AP)
    訂購熱線:400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術支持:techsupport@bioss.com.cn
    說 明 書: 100ul  
    100ul/2980.00元
    大包裝/詢價
    產品編號 bs-13613R-AP
    英文名稱 Rabbit Anti-LRRC6/AP Conjugated antibody
    中文名稱 堿性磷酸酶(AP)標記的富含亮氨酸重復蛋白6抗體
    別    名 Leucine rich repeat containing 6; Leucine rich repeat containing protein 6; Leucine rich testis specific protein; Leucine-rich repeat-containing protein 6; Leucine-rich testis-specific protein; Lrrc6; LRTP; Protein TILB homolog; Testis specific leucine rich repeat protein; Testis-specific leucine-rich repeat protein; TILB_HUMAN; TSLRP.  
    規格價格 100ul/2980元 購買        大包裝/詢價
    說 明 書 100ul  
    研究領域 細胞生物  發育生物學  干細胞  轉錄調節因子  
    抗體來源 Rabbit
    克隆類型 Polyclonal
    交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, )
    產品應用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 54kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human LRRC6
    亞    型 IgG
    純化方法 affinity purified by Protein A
    儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
    產品介紹 background:
    The leucine-rich repeat-containing protein 6 (LRRC6), also designated Leucine-rich testis-specific protein (LRTP), is a 466 amino acid protein that contains 3 LRR repeats and plays a role in spermatogenesis. The gene encoding LRRC6 maps to chromosome 8, which encodes approximately 800 genes. Translocation of portions of chromosome 8 with amplifications of the c-Myc gene are found in some leukemias and lymphomas, and typically associated with a poor prognosis. Chromosome 8 is also associated with Trisomy 8, Pfeiffer syndrome, congenital hypothyroidism and Waardenburg syndrome.

    Function:
    May play a role in dynein arm assembly, hence essential for proper axoneme building for cilia motility.

    Subcellular Location:
    Cytoplasm. Cell projection > cilium.

    Tissue Specificity:
    Expressed predominantly in testis and in nasal epithelial cells.

    DISEASE:
    Defects in LRRC6 are the cause of primary ciliary dyskinesia 19 (CILD19) [MIM:614935]. 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 tilb protein family.
    Contains 1 CS domain.
    Contains 4 LRR (leucine-rich) repeats.
    Contains 1 LRRCT domain.

    Database links:

    Entrez Gene: 23639 Human

    Entrez Gene: 54562 Mouse

    Entrez Gene: 299920 Rat

    SwissProt: Q86X45 Human

    SwissProt: O88978 Mouse

    Unigene: 591865 Human

    Unigene: 244890 Mouse



    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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