Description

  • Product name

    Recombinant Human FH/Fumarase protein (His tag)
    See all FH/Fumarase proteins and peptides
  • Purity

    > 95 % SDS-PAGE.

  • Endotoxin level

    < 1.000 Eu/µg
  • Expression system

    Escherichia coli
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      ASQNSFRIEYDTFGELKVPNDKYYGAQTVRSTMNFKIGGVTERMPTPVIK AFGILKRAAAEVNQDYGLDPKIANAIMKAADEVAEGKLNDHFPLVVWQTG SGTQTNMNVNEVISNRAIEMLGGELGSKIPVHPNDHVNKSQSSNDTFPTA MHIAAAIEVHEVLLPGLQKLHDALDAKSKEFAQIIKIGRTHTQDAVPLTL GQEFSGYVQQVKYAMTRIKAAMPRIYELAAGGTAVGTGLNTRIGFAEKVA AKVAALTGLPFVTAPNKFEALAAHDALVELSGAMNTTACSLMKIANDIRF LGSGPRSGLGELILPENEPGSSIMPGKVNPTQCEAMTMVAAQVMGNHVAV TVGGSNGHFELNVFKPMMIKNVLHSARLLGDASVSFTENCVVGIQANTER INKLMNESLMLVTALNPHIGYDKAAKIAKTAHKNGSTLKETAIELGYLTA EQFDEWVKPKDMLGPK
    • Predicted molecular weight

      51 kDa including tags
    • Amino acids

      45 to 510
    • Tags

      His tag C-Terminus
    • Additional sequence information

      Accession # AAH03108 This product is for the mature full length protein. The transit peptide is not included.

Specifications

Our Abpromise guarantee covers the use of ab219699 in the following tested applications.

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

  • Applications

    SDS-PAGE

  • Form

    Lyophilised
  • Additional notes

     This product was previously labelled as FH

     

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at 4°C (stable for up to 12 months). Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 7.50
    Constituents: 0.87% Sodium chloride, 3% Trehalose, 0.61% Tris

    Lyophilized from 0.22 µm filtered solution.
    Note: 5-10% trehalose is commonly used for freeze drying, and after reconstitution, the trehalose is mostly about 3-5%.

  • Reconstitution
    It is recommended to reconstitute the lyophilized protein in sterile deionized water to a final concentration of 1 mg/ml. Solubilize for 30 to 60 minutes at room temperature with occasional gentle mixing. Carrier protein (0.1% HSA or BSA) is strongly recommended for further dilution and long term storage.

General Info

  • Alternative names

    • FH
    • Fumarase
    • Fumarate hydratase
    • Fumarate hydratase mitochondrial
    • Fumarate hydratase, mitochondrial
    • FUMH_HUMAN
    • HLRCC
    • LRCC
    • MCL
    • MCUL 1
    • MCUL1
    • MS709
    • Multiple hereditary cutaneous leiomyomata
    see all
  • 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

Images

  • SDS-PAGE analysis of DTT-reduced ab219699 stained overnight with Coomassie Blue.

References

ab219699 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab219699.
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