Human Fumarase/FH antibody

Fumarase (Fumarate hytdratase) is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to S-malate and is involved in the tricarboxylic acid (TCA), or Krebs cycle. This enzyme exists in both a cytosolic form and an N-terminal extended mitochondrial form. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension is the same form as in the cytoplasm. Fumarase deficiency can lead to progressive encephalopathy, cerebral atrophy and developmental delay and this enzyme also is thought to act as a tumor suppressor.

Product Specifications

Product Name Alternative

Fumarate hydratase, FH, HLRCC, LRCC, MCL, MCuL1, Fumarate hydratase Fumarase, Fumarate hydratase mitochondrial, MCuL 1, Multiple hereditary cutaneous leiomyomata.

Host

Mouse

Antigen Species

Human

Reactivity

Human

Immunogen

Recombinant human Fumarase (44-510aa) purified from E. coli

Clonality

Monoclonal

Isotype

IgG2a κ

Clone

AT3F6

Conjugation

Unconjugated

Applications

ELISA, WB, ICC/IF, FACS

Purification Method

By protein-A affinity chromatography

Concentration

1 mg/mL (determined by BCA assay)

Additionnal Information

Fumarase, Fumarate hydratase, FH, HLRCC, LRCC, MCL, MCuL1, Fumarate hydratase Fumarase, Fumarate hydratase mitochondrial, MCuL 1, Multiple hereditary cutaneous leiomyomata., ATGA0366-10 µg, ATGA0366-20 µg, ATGA0366-50 µg, ATGA0366-100 µg, ATGA0366-250 µg, ATGA0366-500 µg, ATGA0366-1 mg, ATGA0366-10, ATGA0366-20, ATGA0366-50, ATGA0366-100, ATGA0366-250, ATGA0366-500, ATGA0366-1

References & Citations

Lehtonen R., et al. (2003) J Med Genet. 40 (3) : e19. ; ; Toro JR., et al. (2003) Am J Hum Genet. 73 (1) : 95-106.

Storage Conditions

Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Formulation

Liquid in. Phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerol

Applications Notes

The antibody has been tested by ELISA and Western blot analysis to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results.

Scientific Category

Cell Cycle

NCBI Accession Number

NP_000134

Uniprot Accession Number

P07954

WB Description

The Cell lysates (40ug) were resolved by SDS-PAGE, transferred to PVDF membrane and probed with anti-human Fumarase antibody (1:1000) . Proteins were visualized using a goat anti-mouse secondary antibody conjugated to HRP and an ECL detection system. ; ; Lane 1. : HeLa cell lysate ; ; Lane 2. : LNCap cell lysate ; ; Lane 3. : A549 cell lysate ; ; Lane 4. : HepG2 cell lysate ; ; Lane 5. : 293T cell lysate ; ; Lane 6. : K562 cell lysate ; ; Lane 7. : Jurkat cell lysate

FACS Description

Flow cytometry analysis of Fumarase in HeLa cell line, staining at 2-5ug for 1x106cells. The secondary antibody used goat anti-mouse IgG Alexa fluor 488 conjugate.

IF Description

ICC/IF analysis of Fumarase in A549 cells line, stained with DAPI (Blue) for nucleus staining and monoclonal anti-human Fumarase antibody (1:100) with goat anti-mouse IgG-Alexa fluor 488 conjugate (Green) .