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Anti-Niemann Pick C1 抗体 (ab106534)

Submit a review Q&A (1)References (1)
Western blot - Anti-Niemann Pick C1 antibody (ab106534)
  • Western blot - Anti-Niemann Pick C1 antibody (ab106534)

Key features and details

  • Rabbit polyclonal to Niemann Pick C1
  • Suitable for: ICC/IF, IHC-P, WB
  • Reacts with: Mouse, Human
  • Isotype: IgG

リコンビナント抗体で、ロット間での高い再現性を実現

Product image
Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)
  • 異なるロット間での安定した再現性
  • 容易なスケールアップ
  • 評価試験による特異性の確認済み
  • 倫理基準に準拠 - アニマル・フリーの生産

製品の概要

  • 製品名

    Anti-Niemann Pick C1 antibody
    Niemann Pick C1 一次抗体 製品一覧

  • 製品の詳細

    Rabbit polyclonal to Niemann Pick C1

  • 由来種

    Rabbit

  • 特異性

    Will not cross-react with NPC2

  • アプリケーション

    適用あり: ICC/IF, IHC-P, WBmore details

  • 種交差性

    交差種: Mouse, Human

  • 免疫原

    Synthetic peptide, 16 aa from near the carboxy terminus of human NPC1 (NP_000262).

  • ポジティブ･コントロール

    • WB: HepG2 cell lysate; Human kidney lysate.

  • 特記事項

    The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.

    If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As

アプリケーション

The Abpromise guarantee

Abpromise保証は、 次のテスト済みアプリケーションにおけるab106534の使用に適用されます

アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。

アプリケーション Abreviews 特記事項
ICC/IF
Use at an assay dependent concentration.
IHC-P
Use at an assay dependent concentration.
WB
Use a concentration of 1 - 2 µg/ml. Predicted molecular weight: 142 kDa.
特記事項
ICC/IF
Use at an assay dependent concentration.
IHC-P
Use at an assay dependent concentration.
WB
Use a concentration of 1 - 2 µg/ml. Predicted molecular weight: 142 kDa.

ターゲット情報

  • 機能

    Involved in the intracellular trafficking of cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals.

  • 関連疾患

    Defects in NPC1 are the cause of Niemann-Pick disease type C1 (NPDC1) [MIM:257220]. A lysosomal storage disorder that affects the viscera and the central nervous system. It is due to defective intracellular processing and transport of low-density lipoprotein derived cholesterol. It causes accumulation of cholesterol in lysosomes, with delayed induction of cholesterol homeostatic reactions. Niemann-Pick disease type C1 has a highly variable clinical phenotype. Clinical features include variable hepatosplenomegaly and severe progressive neurological dysfunction such as ataxia, dystonia and dementia. The age of onset can vary from infancy to late adulthood. An allelic variant of Niemann-Pick disease type C1 is found in people with Nova Scotia ancestry. Patients with the Nova Scotian clinical variant are less severely affected.

  • 配列類似性

    Belongs to the patched family.
    Contains 1 SSD (sterol-sensing) domain.

  • ドメイン

    A cysteine-rich N-terminal domain and a C-terminal domain containing a di-leucine motif necessary for lysosomal targeting are critical for mobilization of cholesterol from lysosomes.

  • 翻訳後修飾

    Glycosylated.

  • 細胞内局在

    Late endosome membrane. Lysosome membrane.
  • Information by UniProt

  • 参照データベース

    • 別名

      • Niemann Pick C1 protein precursor antibody
      • Niemann Pick disease, type C1 antibody
      • Niemann-Pick C1 protein antibody
      • NPC antibody
      • NPC1 antibody
      • NPC1_HUMAN antibody
      see all

    画像

    • Western blot - Anti-Niemann Pick C1 antibody (ab106534)
      Western blot - Anti-Niemann Pick C1 antibody (ab106534)
      Anti-Niemann Pick C1 antibody (ab106534) at 1 µg/ml + HepG2 (human liver hepatocellular carcinoma cell line) cell lysate

      Predicted band size: 142 kDa

    • Western blot - Anti-Niemann Pick C1 antibody (ab106534)
      Western blot - Anti-Niemann Pick C1 antibody (ab106534)
      Anti-Niemann Pick C1 antibody (ab106534) at 1 µg/ml + Human kidney tissue lysate at 15 µg

      Predicted band size: 142 kDa

    参考文献 (1)

    ab106534 を使用した論文を発表された方は、こちらまでお知らせください。データシートに掲載させていただきます。

    ab106534 は 1 報の論文で使用されています。

    • Erwood S  et al. Modeling Niemann-Pick disease type C in a human haploid cell line allows for patient variant characterization and clinical interpretation. Genome Res 29:2010-2019 (2019). PubMed: 31754021

    レビューと Q&A

    Show All レビュー Q&A
    レビューを送る 質問を送る

    Question

    Inquiry: Good afternoon, I was wondering if the Anti-Niemann Pick C1 antibody (ab36983) could be usefull to detect the homologous protein in yeast. Indeed, I was looking for a antibody against Ncr1p (NPC1 homologous yeast protein), but it is not available. I've looked for the peptide sequence specific for your antibody, but is not available in the datasheet of this antibody. Hope you could help with this. Thank you so much

    Read More
    Answer

    Thank you for your inquiry.

    Unfortunately, I don't think that any of the https://www.abcam.com/index.html?t=158346&pt=1 we have in our catalog is suitable for the detection of the yeast Niemann-Pick type C-related protein 1 (http://www.uniprot.org/uniprot/Q12200).
    I compared the immunogen sequence of each antibodywith the yeast protein and the maximum score is only 35%.

    I would like to recommend checking the Biocompare website which has an excellent antibody search facility that includes many suppliers. The link is: http://www.biocompare.com

    I hope this information is nevertheless helpful to you. Please do not hesitate to contact me if you have any further questions in this regard.

    Read More

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