概述

  • 产品名称Anti-CLN8抗体
  • 描述
    兔多克隆抗体to CLN8
  • 经测试应用适用于: WB, ELISAmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Horse, Guinea pig, Cat, Dog
  • 免疫原

    Synthetic peptide corresponding to N terminal amino acids 1-49 (MNPASDGGTS ESIFDLDYAS WGIRSTLMVA GFVFYLGVFV VCHQLSSSLN) , of Human CLN8 (NP_061764)

  • 阳性对照
    • HeLa cell lysate

性能

应用

Our Abpromise guarantee covers the use of ab82759 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
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 33 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
ELISA Use at an assay dependent concentration.

ELISA titre using peptide based assay: 1/12500.

靶标

  • 功能Could play a role in cell proliferation during neuronal differentiation and in protection against cell death.
  • 疾病相关Defects in CLN8 are the cause of neuronal ceroid lipofuscinosis type 8 (CLN8) [MIM:600143]. A form of neuronal ceroid lipofuscinosis with onset in childhood. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment patterns observed most often in neuronal ceroid lipofuscinosis type 8 comprise mixed combinations of granular, curvilinear, and fingerprint profiles.
    Defects in CLN8 are the cause of neuronal ceroid lipofuscinosis type 8 Northern epilepsy variant (CLN8NE) [MIM:610003]. A form of neuronal ceroid lipofuscinosis clinically characterized by epilepsy that presents between 5 and 10 years of age with frequent tonic-clonic seizures followed by progressive mental retardation. Visual loss is not a prominent feature. Intracellular accumulation of autofluorescent material results in curvilinear and granular profiles on ultrastructural analysis.
  • 序列相似性Contains 1 TLC (TRAM/LAG1/CLN8) domain.
  • 翻译后修饰Does not seem to be N-glycosylated.
  • 细胞定位Endoplasmic reticulum membrane. Endoplasmic reticulum-Golgi intermediate compartment membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • C8orf61 antibody
    • Ceroid-lipofuscinosis, neuronal 8 (epilepsy, progressive with mental retardation) antibody
    • Cln8 antibody
    • CLN8 gene antibody
    • CLN8_HUMAN antibody
    • EPMR antibody
    • Protein CLN8 antibody
    see all

Anti-CLN8 antibody 图像

  • Anti-CLN8 antibody (ab82759) at 1 µg/ml + HeLa cell lysate at 10 µg with skim milk/ PBS at 5 %

    Secondary
    HRP conjugated anti-Rabbit IgG at 1/50000 dilution

    Predicted band size : 33 kDa
    Observed band size : 43 kDa (why is the actual band size different from the predicted?)

Anti-CLN8 antibody (ab82759)参考文献

ab82759 has not yet been referenced specifically in any publications.

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