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RSPH4A Rabbit pAb(bs-11472R)-5

0ul/100ul/200ul
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  • ¥1180 - 2800
  • Bioss已认证
  • bs-11472R
  • 2025年10月24日
  • 产品信息以Bioss网站为准
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      50ul/100ul/200ul

    规格:50ul产品价格:¥1180.0
    规格:100ul产品价格:¥1980.0
    规格:200ul产品价格:¥2800.0
    产品编号bs-11472R
    英文名称RSPH4A Rabbit pAb
    中文名称Kartagener综合征相关蛋白RSHL3抗体
    英文别名CILD11; dJ412I7.1; Radial spoke head protein 4 homolog A; Radial spoke head-like protein 3; RSH4A_HUMAN; RSHL3; Rsph4a; RSPH6B; A230081C05.
    产品应用WB=1:500-2000

    Not yet tested in other applications.
    Optimal working dilutions must be determined by the end user.

    交叉反应Human, Mouse (Rat, Chicken, Dog, Pig, Cow, Horse, Sheep)
    抗体来源Rabbit
    免疫原KLH conjugated synthetic peptide derived from human RSPH4A/RSHL3
    亚型IgG
    性状Liquid
    纯化方法affinity purified by Protein A
    克隆类型Polyclonal
    理论分子量81 kDa
    浓度1mg/ml
    储存液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    研究领域

    Neuroscience > Neurology process > Growth and Development > Axonal Guidance Proteins

    Signal Transduction > Cytoskeleton / ECM > Cytoskeleton > Motor Proteins > Dynein

    亚细胞定位Cytoplasm; cytoskeleton; cilium axoneme. Radial spoke.
    组织特异性Defects in RSPH4A are the cause of primary ciliary dyskinesia type 11 (CILD11) [MIM:612649]. CILD is an autosomal recessive disorder characterized by axonemal 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 situs inversus, due to dysfunction of monocilia at the embryonic node and randomization of left-right body asymmetry. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome.
    相似性Belongs to the flagellar radial spoke RSP4/6 family.
    功能Probable component of the axonemal radial spoke head. Radial spokes are regularly spaced along cilia, sperm and flagella axonemes. They consist of a thin stalk which is attached to a subfiber of the outer doublet microtubule, and a bulbous head which is attached to the stalk and appears to interact with the projections from the central pair of microtubules.
    保存条件Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    背景资料RSHL3 is predicted to be a component of the radial spoke head based on homology with proteins in the biflagellate alga Chlamydomonas reinhardtii and other ciliates. RSHL3 (radial spoke head-like protein 3), also known as radial spoke head protein 4 homolog A, is a 716 amino acid protein that belongs to the flagellar radial spoke RSP4/6 family. Mutations in the RSHL3 gene cause primary ciliary dyskinesia 1, a disease arising from dysmotility of motile cilia and sperm. Existing as three alternatively spliced isoforms, the RSHL3 gene contains 6 exons, is conserved in chimpanzee, dog, cow, mouse, rat, chicken, zebrafish, fruit fly and P.falciparum, and maps to human chromosome 6q22.1.

     

    应用推荐稀释比例
    {WB}{1:500-2000}

     

    产品细节图片1
    Sample:
    Lung (Mouse) Lysate at 40 ug
    A431(Human) Cell Lysate at 30 ug
    Testis (Mouse) Lysate at 40 ug
    Primary: Anti-RSPH4A  (bs-11472R) at 1/1000 dilution
    Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
    Predicted band size: 81 kD
    Observed band size: 81 kD
    产品细节图片2
    Sample:
    Trachea (Mouse) Lysate at 40 ug
    Primary: Anti-RSPH4A  (bs-11472R) at 1/1000 dilution
    Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
    Predicted band size: 81 kD
    Observed band size: 81 kD

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    RSPH4A Rabbit pAb(bs-11472R)-50ul/100ul/200ul
    ¥1180 - 2800