概述

  • 产品名称Anti-FH抗体
    参阅全部 FH 一抗
  • 描述
    山羊多克隆抗体to FH
  • 经测试应用适用于: Sandwich ELISA, WB, ELISAmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Horse, Cow, Dog, Pig, Drosophila melanogaster, Zebrafish, a wide range of other species, Cynomolgus Monkey, Chlamydia trachomatis, Orangutan
  • 免疫原

    Synthetic peptide:

    C-HPNDHVNKSQSSND

    , corresponding to internal sequence amino acids 176-189 of Human FH

  • 阳性对照
    • Human Kidney, Liver and Lung lysates

性能

应用

Our Abpromise guarantee covers the use of ab62825 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
Sandwich ELISA Use a concentration of 1 - 2 µg/ml. Used as a reporter to detect recombinant Fumarase
WB Use a concentration of 0.01 - 0.03 µg/ml. Detects a band of approximately 48 kDa (predicted molecular weight: 55 kDa).
ELISA 1/64000.

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

  • Anti-FH antibody (ab62825) at 0.01 µg/ml + Human Kidney lysate in RIPA buffer at 35 µg/ml

    Predicted band size : 55 kDa
    Observed band size : 48 kDa (why is the actual band size different from the predicted?)


  • Predicted band size : 55 kDa

Anti-FH antibody (ab62825)参考文献

This product has been referenced in:
  • Kienhöfer J  et al. Association of mitochondrial antioxidant enzymes with mitochondrial DNA as integral nucleoid constituents. FASEB J 23:2034-44 (2009). WB ; Human . Read more (PubMed: 19228881) »

See 1 Publication for this product

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