Anti-CTNS抗体(ab64175)

概述

  • 产品名称Anti-CTNS抗体
    参阅全部 CTNS 一抗
  • 描述
    兔多克隆抗体to CTNS
  • 经测试应用适用于: ELISA, IHC-Pmore details
  • 种属反应性
    与反应: Human
  • 免疫原

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

  • 阳性对照
    • Human skeletal muscle tissue.

性能

应用

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.

应用 Ab评论 说明
ELISA 1/1 - 1/1000.
IHC-P Use a concentration of 1.25 µg/ml.

靶标

  • 功能Thought to transport cystine out of lysosomes.
  • 组织特异性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).
  • 疾病相关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.
  • 序列相似性Belongs to the cystinosin family.
    Contains 2 PQ-loop domains.
  • 细胞定位Lysosome membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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 图像

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

Anti-CTNS antibody (ab64175)参考文献

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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