概述

  • 产品名称Anti-AFG3L2抗体
    参阅全部 AFG3L2 一抗
  • 描述
    兔多克隆抗体to AFG3L2
  • 经测试应用适用于: ELISA, WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Rabbit, Horse, Chicken, Guinea pig, Cow, Cat, Dog, Saccharomyces cerevisiae, Caenorhabditis elegans, Drosophila melanogaster, Zebrafish
  • 免疫原

    Synthetic peptide: VNFLKNPKQY QDLGAKIPKG AILTGPPGTG KTLLAKATAG EANVPFITVS , corresponding to a region within internal sequence amino acids 324 - 373 of Human AFG3L2 (NP_006787)

  • 阳性对照
    • HeLa cell lysate

性能

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应用

Our Abpromise guarantee covers the use of ab83418 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 Use at an assay dependent concentration.

ELISA titre using peptide based assay: 1/312500.

WB Use a concentration of 1 µg/ml. Predicted molecular weight: 89 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

靶标

  • 功能ATP-dependent protease which is essential for axonal development.
  • 组织特异性Ubiquitous. Highly expressed in the cerebellar Purkinje cells.
  • 疾病相关Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28) [MIM:610246]. It is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA28 is an autosomal dominant cerebellar ataxia (ADCA) with a slow progressive course and no evidence of sensory involvement or cognitive impairment.
    Defects in AFG3L2 are the cause of spastic ataxia autosomal recessive type 5 (SPAX5) [MIM:614487]. A neurodegenerative disorder characterized by early onset spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy.
  • 序列相似性In the N-terminal section; belongs to the AAA ATPase family.
    In the C-terminal section; belongs to the peptidase M41 family.
  • 细胞定位Mitochondrion membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • AFG3 (ATPase family gene 3, yeast) like 2 antibody
    • AFG3 ATPase family gene 3 like 2 (yeast) antibody
    • AFG3 ATPase family gene 3 like 2 antibody
    • AFG3 like protein 2 antibody
    • AFG3-like protein 2 antibody
    • AFG32_HUMAN antibody
    • AFG3L2 antibody
    • ATPase family gene 3 like 2 antibody
    • ATPase family gene 3 yeast antibody
    • EC 3.4.24.- antibody
    • FLJ25993 antibody
    • Paraplegin like protein antibody
    • Paraplegin-like protein antibody
    • SCA28 antibody
    • Spinocerebellar ataxia 28 antibody
    see all

Anti-AFG3L2 antibody 图像

  • Anti-AFG3L2 antibody (ab83418) at 1 µg/ml (in 5% skim milk / PBS buffer) + HeLa cell lysate at 10 µg

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

    Predicted band size : 89 kDa
    Observed band size : 90 kDa (why is the actual band size different from the predicted?)
    Additional bands at : 45 kDa. We are unsure as to the identity of these extra bands.

Anti-AFG3L2 antibody (ab83418)参考文献

ab83418 has not yet been referenced specifically in any publications.

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