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    LRRC6 Rabbit pAb (bs-13613R)  
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    產品編號 bs-13613R
    英文名稱 LRRC6 Rabbit pAb
    中文名稱 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.  
    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
    克 隆 號
    交叉反應 Human,Mouse (predicted: Rat,Pig,Sheep,Cow,Dog,Horse)
    產品應用 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 54 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 LRRC6: 201-300/466 
    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
    產品介紹 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.

    SWISS:
    Q86X45

    Gene ID:
    23639

    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



    產品圖片
    Sample: Lane 1: Testis (Mouse) Lysate at 40 ug Lane 2: A549 (Human) Cell Lysate at 30 ug Primary: Anti-LRRC6 (bs-13613R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 54 kD Observed band size: 54 kD
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