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    RBM28 Rabbit pAb (bs-19769R)  
    訂購熱線(xiàn):400-901-9800
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    訂購QQ:  400-901-9800
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    50ul/1180.00元
    100ul/1980.00元
    200ul/2800.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-19769R
    英文名稱(chēng) RBM28 Rabbit pAb
    中文名稱(chēng) RNA結合蛋白28抗體
    別    名 2810480G15Rik; FLJ10377; RBM 28; RBM28; RBM28_HUMAN; RNA binding motif protein 28; RNA binding protein 28; RNA-binding motif protein 28; RNA-binding protein 28.  
    研究領(lǐng)域 細胞生物  發(fā)育生物學(xué)  神經(jīng)生物學(xué)  結合蛋白  表觀(guān)遺傳學(xué)  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 (predicted: Human,Mouse,Rabbit,Dog,Horse)
    產(chǎn)品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 85 kDa
    檢測分子量
    細胞定位 細胞核 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human RBM28: 601-700/759 
    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(chǎn)品介紹 The protein encoded by this gene is a specific nucleolar component of the spliceosomal small nuclear ribonucleoprotein (snRNP)complexes . It specifically associates with U1, U2, U4, U5, and U6 small nuclear RNAs (snRNAs), possibly coordinating their transition through the nucleolus. Mutation in this gene causes alopecia, progressive neurological defects, and endocrinopathy (ANE syndrome), a pleiotropic and clinically heterogeneous disorder. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]

    Function:
    Nucleolar component of the spliceosomal ribonucleoprotein complexes.

    Subcellular Location:
    Nucleus > nucleolus.

    Tissue Specificity:
    Ubiquitously expressed.

    DISEASE:
    Defects in RBM28 are the cause of alopecia neurologic defects and endocrinopathy syndrome (ANES) [MIM:612079]. Affected individuals have hair loss of variable severity, ranging from complete alopecia to near-normal scalp hair with absence of body hair. All have moderate to severe mental retardation, progressive motor deterioration and central hypogonadotropic hypogonadism with delayed or absent puberty and central adrenal insufficiency. Additional features included short stature, microcephaly, gynecomastia, pigmentary anomalies, hypodontia, kyphoscoliosis, ulnar deviation of the hands, and loss of subcutaneous fat.

    Similarity:
    Contains 4 RRM (RNA recognition motif) domains.

    SWISS:
    Q9NW13

    Gene ID:
    55131

    Database links:

    Entrez Gene: 475203 Dog

    Entrez Gene: 100071725 Horse

    Entrez Gene: 55131 Human

    Entrez Gene: 100338231 Rabbit

    Omim: 612074 Human

    SwissProt: Q9NW13 Human

    Unigene: 274263 Human



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