概述

  • 产品名称Anti-FH抗体
    参阅全部 FH 一抗
  • 描述
    小鼠单克隆抗体to FH
  • 经测试应用适用于: WB, ICC/IFmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Recombinant full length protein, corresponding to amino acids 33-511 of Human FH

性能

应用

Our Abpromise guarantee covers the use of ab58232 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 - 5 µg/ml. Predicted molecular weight: 55 kDa.
ICC/IF Use at an assay dependent concentration. PubMed: 22867999

靶标

  • 功能Also acts as a tumor suppressor.
  • 通路Carbohydrate metabolism; tricarboxylic acid cycle; (S)-malate from fumarate: step 1/1.
  • 疾病相关Defects in FH are the cause of fumarase deficiency (FHD) [MIM:606812]; also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia.
    Defects in FH are the cause of multiple cutaneous and uterine leiomyomata (MCUL1) [MIM:150800]. MCUL1 is an autosomal dominant condition in which affected individuals develop benign smooth muscle tumors (leiomyomata) of the skin. Affected females also usually develop leiomyomata of the uterus (fibroids).
    Defects in FH are the cause of hereditary leiomyomatosis and renal cell cancer (HLRCC) [MIM:605839].
  • 序列相似性Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.
  • 细胞定位Cytoplasm and Mitochondrion.
  • Information by UniProt
  • 数据库链接
  • 别名
    • FH antibody
    • Fumarase antibody
    • Fumarate hydratase antibody
    • Fumarate hydratase mitochondrial antibody
    • Fumarate hydratase, mitochondrial antibody
    • FUMH_HUMAN antibody
    • HLRCC antibody
    • LRCC antibody
    • MCL antibody
    • MCUL 1 antibody
    • MCUL1 antibody
    • MS709 antibody
    • Multiple hereditary cutaneous leiomyomata antibody
    see all

Anti-FH antibody 图像



  • Predicted band size : 55 kDa
    FH antibody (ab58232) at 1ug/lane + HeLa cell lysate at 25ug/lane.
  • Immunofluorescence analysis of UOK268 cells, staining FH with ab58232.

    Cells were fixed in formaldehyde and permeabilized with 0.2% Triton X-100. Samples were incubated with primary antibody (1/200) for 1 hour at room temperature. An AlexaFluor®488-conjugated goat anti-mouse IgG (1/500) was used as the secondary antibody.

Anti-FH antibody (ab58232)参考文献

This product has been referenced in:
  • Beernink PT  et al. Heterogeneity in rhesus macaque complement factor H binding to meningococcal factor H binding protein (FHbp) informs selection of primates to assess immunogenicity of FHbp-based vaccines. Clin Vaccine Immunol 21:1505-11 (2014). Read more (PubMed: 25185576) »
  • Yang Y  et al. A novel fumarate hydratase-deficient HLRCC kidney cancer cell line, UOK268: a model of the Warburg effect in cancer. Cancer Genet 205:377-90 (2012). ICC/IF ; Human . Read more (PubMed: 22867999) »

See all 4 Publications for this product

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