AHI1 Rabbit pAb(bs-7854R)-50ul/100ul/200ul

AHI1 Rabbit pAb(bs-7854R)-50ul

/100ul/200ul
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  • ¥1180 - 2800
  • Bioss已认证
  • bs-7854R
  • 2025年10月16日
  • 产品信息以Bioss网站为准
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    • 规格

      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-7854R
    英文名称AHI1 Rabbit pAb
    中文名称白血病相关蛋白AHI1抗体
    英文别名Abelson helper integration site 1 protein homolog; Abelson helper integration site 1; Abelson helper integration site; AHI 1; AHI-1; Ahi1; AHI1_HUMAN; Contatins SH3 and WD40 domains; JBTS3; Jouberin; ORF1.
    产品应用WB=1:500-2000, IHC-P=1:100-500, IHC-F=1:100-500, IF=1:100-500, ELISA=1:5000-10000

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

    交叉反应 (Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep)
    抗体来源Rabbit
    免疫原KLH conjugated synthetic peptide derived from human AHI1
    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137 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

    Cell Biology > Cell Cycle > Cell differentiation

    亚基Part of the tectonic-like complex (also named B9complex). Interacts with MKS1 (By similarity). Interacts withNPHP1.
    亚细胞定位Cytoplasm, cytoskeleton, cilium basal body(By similarity). Cell junction, adherens junction.
    组织特异性Highly expressed in the most primitive normalhematopoietic cells. Expressed in brain, particularly in neuronsthat give rise to the crossing axons of the corticospinal tract andsuperior cerebellar peduncles. Expressed in kidney (renalcollecting duct cells) (at protein level).
    相似性Contains 1 SH3 domain.
    Contains 7 WD repeats.
    功能Component of the tectonic-like complex, a complexlocalized at the transition zone of primary cilia and acting as abarrier that prevents diffusion of transmembrane proteins betweenthe cilia and plasma membranes (By similarity).
    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Highly expressed in the most primitive normal hematopoietic cells. Expressed in brain, particularly in neurons that give rise to the crossing axons of the corticospinal tract and superior cerebellar peduncles. Expressed in kidney (renal collecting duct cells) (at protein level).Involvement in disease:Defects in AHI1 are the cause of Joubert syndrome type 3 (JBTS3) . JBTS is an autosomal recessive disorder presenting with cerebellar ataxia, oculomotor apraxia, hypotonia, neonatal breathing abnormalities and psychomotor delay. Neuroradiologically, it is characterized by cerebellar vermian hypoplasia/aplasia, thickened and reoriented superior cerebellar peduncles, and an abnormally large interpeduncular fossa, giving the appearance of a molar tooth on transaxial slices (molar tooth sign). Additional variable features include retinal dystrophy and renal disease. JBTS3 shows minimal extra central nervous system involvement and appears not to be associated with renal dysfunction.

     

    应用推荐稀释比例
    {WB}{1:500-2000}
    {IHC-P}{1:100-500}
    {IHC-F}{1:100-500}
    {IF}{1:100-500}
    {ELISA}{1:5000-10000}

     

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    ¥1180 - 2800