Recombinant
RabMAb

Recombinant Anti-FH/Fumarase antibody [EPR11647(2)(B)] - BSA and Azide free (ab250784)

Overview

  • Product name

    Anti-FH/Fumarase antibody [EPR11647(2)(B)] - BSA and Azide free
    See all FH/Fumarase primary antibodies
  • Description

    Rabbit monoclonal [EPR11647(2)(B)] to FH/Fumarase - BSA and Azide free
  • Host species

    Rabbit
  • Tested applications

    Suitable for: Flow Cyt, ICC/IF, WBmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide within Human FH/Fumarase aa 1-100 (N terminal). The exact sequence is proprietary.
    Database link: P07954

  • General notes

    ab250784 is the carrier-free version of ab184945 This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.

     

    Our carrier-free formats are supplied in a buffer free of BSA, sodium azide and glycerol for higher conjugation efficiency.

    Use our conjugation kits  for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.

    Ab250784 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.

    Maxpar® is a trademark of Fluidigm Canada Inc.

    This product was previously labelled as FH

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab250784 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
Flow Cyt Use at an assay dependent concentration.
ICC/IF Use at an assay dependent concentration.
WB Use at an assay dependent concentration. Detects a band of approximately 49 kDa (predicted molecular weight: 55 kDa).

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

References

ab250784 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab250784.
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Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
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