製品の概要

  • 製品名Anti-CTNS antibody
    CTNS 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to CTNS
  • アプリケーション適用あり: ELISA, IHC-Pmore details
  • 種交差性
    交差種: Human
  • 免疫原

    Synthetic peptide conjugated to KLH, corresponding to amino acids 268-285 of Human CTNS

  • ポジティブ・コントロール
    • Human skeletal muscle tissue.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab64175 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
ELISA 1/1 - 1/1000.
IHC-P Use a concentration of 1.25 µg/ml.

ターゲット情報

  • 機能Thought to transport cystine out of lysosomes.
  • 組織特異性Strongly expressed in pancreas, kidney (adult and fetal) and in skeletal muscle. Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (adult and fetal).
  • 関連疾患Cystinosis, nephropathic type (CTNS) [MIM:219800]: A form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. The classical nephropathic form has onset in the first year of life and is characterized by a polyuro-polydipsic syndrome, marked height-weight growth delay, generalized impaired proximal tubular reabsorptive capacity, with severe fluid-electrolyte balance alterations, renal failure, ocular symptoms and other systemic complications. Note=The disease is caused by mutations affecting the gene represented in this entry.
    Cystinosis, adult, non-nephropathic type (CTNSANN) [MIM:219750]: A form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. Cystinosis adult non-nephropathic type is characterized by ocular features and a benigne course. Patients manifest mild photophobia due to conjunctival and corneal cystine crystals. Note=The disease is caused by mutations affecting the gene represented in this entry.
    Cystinosis, late-onset juvenile or adolescent nephropathic type (CTNSJAN) [MIM:219900]: A form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. Late-onset juvenile or adolescent nephropathic cystinosis is an intermediated form, manifesting first at age 10 to 12 years with proteinuria due to glomerular damage rather than with the manifestations of tubular damage that occur first in infantile cystinosis. There is no excess amino aciduria and stature is normal. Photophobia, late development of pigmentary retinopathy, and chronic headaches are features. Note=The disease is caused by mutations affecting the gene represented in this entry.
  • 配列類似性Belongs to the cystinosin family.
    Contains 2 PQ-loop domains.
  • 細胞内局在Lysosome membrane.
  • Information by UniProt
  • 参照データベース
  • 別名
    • ctns antibody
    • CTNS LSB antibody
    • CTNS_HUMAN antibody
    • Cystinosin antibody
    • Cystinosin, lysosomal cystine transporter antibody
    • cystinosis, nephropathic antibody
    • PQLC4 antibody
    see all

Anti-CTNS antibody 画像

  • ab64175 at 1.25µg/ml staining CTNS in human skeletal muscle by Immunohistochemistry, Formalin-fixed, paraffin-embedded tissue.

Anti-CTNS antibody (ab64175) 使用論文

ab64175 has not yet been referenced specifically in any publications.

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We have not tested the CTNS antibody ab64175 in immunoprecipitation at this time, so we are not sure whether it will work or not. We do not have any other CTNS antibodies, unfortunatel...

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