Date published: 2025-9-7

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NAGLU 抗体 (54-G): sc-130383

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说明书
  • NAGLU 抗体 54-G 是小鼠单克隆 IgG2a (kappa轻链),NAGLU抗体,规格为100 µg/ml
  • 针对部分重组蛋白,其氨基酸序列在NAGLU的human起源的644-743范围内
  • 推荐用于 mouse, rat 和 human 来源的NAGLU WB 和 IP检测
  • 关于如何获取NAGLU (54-G): sc-130383免费10 µg小样,联系我们技术服务部门 (或者您当地的代理商)了解详情。
  • 目前,我们还没有完成NAGLU 抗体 (54-G)的首选二抗检测试剂的鉴定。这项工作正在进行中。

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    NAGLU抗体(54-G)是一种IgG2a κ小鼠单克隆NAGLU抗体(也称为NAGLU抗体),可通过WB和IP检测小鼠、大鼠和人类来源的NAGLU蛋白。NAGLU抗体(54-G)为非偶联的抗NAGLU抗体。NAGLU(N-乙酰-α-葡萄糖苷酶),也称为NAG、UFHSD1、MPS3B或MPS-IIIB,是一种由743个氨基酸组成的蛋白质,既以单体形式存在,也以同源二聚体形式存在。NAGLU在卵巢、肝脏、睾丸、前列腺、肺、结肠、肾脏、脾脏、胎盘和外周血白细胞中表达,参与硫酸乙酰肝素(HS)的降解,特别是催化N-乙酰-α-D-葡糖胺中的末端N-乙酰-D-葡糖胺残基的水解。编码NAGLU的基因缺陷是引起IIIB型粘多糖病(MPS-IIIB,也称为Sanfilippo综合征B型)的原因。MPS-IIIB是一种常染色体隐性遗传病,患者体内无法降解HS,导致HS在溶酶体和尿液中积累,造成智力衰退,最终导致死亡。

    仅限研究使用。不适用于诊断和治疗用途。

    Alexa Fluor® 是Molecular Probes Inc., OR., USA的商标

    LI-COR®和 Odyssey® 是LI-COR Biosciences的注册商标

    NAGLU 抗体 (54-G) 参考文献:

    1. 桑菲利波 B 型综合征(粘多糖病 III B):等位基因的异质性与临床表型的广泛性相对应。  |  Weber, B., et al. 1999. Eur J Hum Genet. 7: 34-44. PMID: 10094189
    2. 黏多醣症 IIIA 型和 IIIB 型的分子遗传学:诊断, 临床和生物学意义。  |  Yogalingam, G. and Hopwood, JJ. 2001. Hum Mutat. 18: 264-81. PMID: 11668611
    3. 桑菲利波 B 型综合征:来自日本冲绳群岛的五名α-N-乙酰葡萄糖苷酶基因 R565P 同源突变患者。  |  Chinen, Y., et al. 2005. J Hum Genet. 50: 357-359. PMID: 15933803
    4. B 型桑菲利波综合征小鼠模型感官, 运动和行为缺陷的发展。  |  Heldermon, CD., et al. 2007. PLoS One. 2: e772. PMID: 17712420
    5. 从结构和机理上洞察粘多糖病 IIIB 的发病基础。  |  Ficko-Blean, E., et al. 2008. Proc Natl Acad Sci U S A. 105: 6560-5. PMID: 18443291
    6. 在B型桑菲洛综合征细胞模型中,NAGLU-IGF2和未修饰的NAGLU的差异摄取。  |  Prill, H., et al. 2019. Mol Ther Methods Clin Dev. 14: 56-63. PMID: 31309128
    7. 血管内皮细胞中 NAGLU 的下调会增加溶酶体的异常积累,是早期动脉粥样硬化的预测性生物标记物。  |  Xing, C., et al. 2021. Front Cell Dev Biol. 9: 797047. PMID: 35155448
    8. 与新型显性NAGLU突变相关的早发性运动神经元病。  |  Lopergolo, D., et al. 2023. Neurol Sci. 44: 1415-1418. PMID: 36648562
    9. 桑菲利波综合征 B 型的分子基础  |  Zhao, HG., et al. 1996. Proc Natl Acad Sci U S A. 93: 6101-5. PMID: 8650226
    10. 圣菲利波 B 综合征(粘多糖病 III B)相关基因的克隆和表达。  |  Weber, B., et al. 1996. Hum Mol Genet. 5: 771-7. PMID: 8776591
    11. 桑菲利波综合征 B 型的 NAGLU 基因突变。  |  Schmidtchen, A., et al. 1998. Am J Hum Genet. 62: 64-9. PMID: 9443878
    12. 黏多醣症 IIIB 型(桑菲利波 B):18 种新型α-N-乙酰葡糖苷酶基因突变的鉴定。  |  Bunge, S., et al. 1999. J Med Genet. 36: 28-31. PMID: 9950362

    订购信息

    产品名称产品编号规格价格数量收藏夹

    NAGLU 抗体 (54-G)

    sc-130383
    100 µg/ml
    $333.00

    NAGLU (54-G) sc-130383 can be used as bio marker for renal function as beta N-acetyl-glucosaminidase

    Asked by: sayed abd allateef
    Thank you for your question. This antibody recognizes NAGLU (N-acetyl-alpha-glucosaminidase), also known as NAG, UFHSD1, MPS3B or MPS-IIIB. We do not have any information about whether or not this antigen is a suitable bio marker for renal function.
    Answered by: Technical Support
    Date published: 2019-08-17

    I am using NAGLU (54-G): sc-130383 antibody for immunoprecipitation, how much antibody should I use?

    Asked by: Trav11
    Thank you for your question. We recommend using 1–10 µl (i.e., 0.2–2 µg) of antibody with 1 ml of cell lysate. However, optimal antibody concentration should be determined by titration. Please find our full immunoprecipitation protocol here: https://www.scbt.com/scbt/resources/protocols/immunoprecipitation-western-blots
    Answered by: Technical Support
    Date published: 2017-03-24
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    Rated 5 out of 5 by from Good for Western BlotThis antibody detects a band in Jurkat whole cell lysate by Western Blot. -SCBT QC
    Date published: 2023-09-14
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    NAGLU 抗体 (54-G) is rated 4.5 out of 5 by 2.
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