Description

  • Product name

    Recombinant human FH/Fumarase protein
    See all FH/Fumarase proteins and peptides
  • Biological activity

    Specific activity is > 1.0 unit/mg, and is defined as the amount of enzyme that cleaves 1 µmole of L-Malate to Fumarate per minute at pH 7.5 at 25°C
  • 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

      MASQNSFRIE YDTFGELKVP NDKYYGAQTV RSTMNFKIGG VTERMPTPVI KAFGILKRAA AEVNQDYGLD PKIANAIMKA ADEVAEGKLN DHFPLVVWQT GSGTQTNMNV NEVISNRAIE MLGGELGSKI PVHPNDHVNK SQSSNDTFPT AMHIAAAIEV HEVLLPGLQK LHDALDAKSK EFAQIIKIGR THTQDAVPLT LGQEFSGYVQ QVKYAMTRIK AAMPRIYELA AGGTAVGTGL NTRIGFAEKV AAKVAALTGL PFVTAPNKFE ALAAHDALVE LSGAMNTTAC SLMKIANDIR FLGSGPRSGL GELILPENEP GSSIMPGKVN PTQCEAMTMV AAQVMGNHVA VTVGGSNGHF ELNVFKPMMI KNVLHSARLL GDASVSFTEN CVVGIQANTE RINKLMNESL MLVTALNPHI GYDKAAKIAK TAHKNGSTLK ETAIELGYLT AEQFDEWVKP KDMLGPK
    • Predicted molecular weight

      50 kDa
    • Amino acids

      44 to 510

Specifications

Our Abpromise guarantee covers the use of ab168013 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

    Functional Studies

  • Form

    Liquid
  • Additional notes

    Centrifuge the vial prior to opening

     This product was previously labelled as FH

     

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 8.00
    Constituent: 0.32% Tris HCl

    This product is an active protein and may elicit a biological response in vivo, handle with caution.

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

  • 15% SDS-PAGE analysis of 3µg ab168013.

References

ab168013 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab168013.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

Sign up