Overview

  • Product name
  • Description
    Rabbit polyclonal to FH
  • Tested applications
    Suitable for: WB, IHC-P, ICC/IFmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Human
  • Immunogen

    Recombinant fragment within Human FH aa 89-365. The exact sequence is proprietary.
    Sequence:

    PTPVIKAFGILKRAAAEVNQDYGLDPKIANAIMKAADEVAEGKLNDHFPL VVWQTGSGTQTNMNVNEVISNRAIEMLGGELGSKIPVHPNDHVNKSQSSN DTFPTAMHIAAAIEVHEVLLPGLQKLHDALDAKSKEFAQIIKIGRTHTQD AVPLTLGQEFSGYVQQVKYAMTRIKAAMPRIYELAAGGTAVGTGLNTRIG FAEKVAAKVAALTGLPFVTAPNKFEALAAHDALVELSGAMNTTACSLMKI ANDIRFLGSGPRSGLGELILPENEPGS


    Database link: P07954

  • Positive control
    • Human liver tissue; HeLa cells; rat and mouse liver tissue lysates; 293T and A431 cell lysates.

Properties

Applications

Our Abpromise guarantee covers the use of ab191367 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/1000 - 1/10000. Predicted molecular weight: 54 kDa.
IHC-P Use a concentration of 7.5 µg/ml.
ICC/IF 1/100 - 1/1000.

Target

  • Function
    Also acts as a tumor suppressor.
  • Pathway
    Carbohydrate metabolism; tricarboxylic acid cycle; (S)-malate from fumarate: step 1/1.
  • Involvement in disease
    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].
  • Sequence similarities
    Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.
  • Cellular localization
    Cytoplasm and Mitochondrion.
  • Information by UniProt
  • Database links
  • Alternative names
    • 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

Images

  • All lanes : Anti-FH antibody (ab191367) at 1/5000 dilution

    Lane 1 : 293T cell lysate
    Lane 2 : A431 cell lysate

    Lysates/proteins at 30 µg per lane.


    Predicted band size : 54 kDa

    10% SDS Page

  • Anti-FH antibody (ab191367) at 1/1000 dilution + mouse liver lysate at 50 µg

    Predicted band size : 54 kDa

    10% SDS Page

  • Anti-FH antibody (ab191367) at 1/5000 dilution + rat liver tissue lysate at 50 µg

    Predicted band size : 54 kDa

    10% SDS Page

  • Immunohistochemical analysis of formalin fixed, paraffin embedded Human liver tissue labeling FH using ab191367 at 7.5 µg/ml.

  • Immunofluorescence analysis of methanol-fixed HeLa cells labeling FH using ab191367 at 1/200 dilution. Bottom image shows Hoechst 33342 counterstain.

References

ab191367 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab191367.
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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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