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Recombinant human Fumarase/FH protein

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. Recombinant Fumarase was expressed in E. coli and was purified by conventional chromatography techniques.

Product Specifications

Specifications

Specific activity is > 25 unit/mg, and is defined as the amount of enzyme that cleaves 1umole of L-Malate to Fumarate per minute at pH 7.5 at 37C.

Product Name Alternative

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

Expression System

E.coli

Antigen Species

Human

Tag

Non-Tagged

Applications

SDS-PAGE, Enzyme Activity

Concentration

1 mg/mL (determined by Bradford assay)

Endotoxin

< 1 EU per 1ug of protein (determined by LAL method)

Purity

> 95% by SDS-PAGE

Bioactivity

Specific activity is > 25 unit/mg, and is defined as the amount of enzyme that cleaves 1umole of L-Malate to Fumarate per minute at pH 7.5 at 37C.

Molecular Weight

50.2 kDa (467aa) confirmed by MALDI-TOF

Additionnal Information

Fumarase, Fumarate hydratase, FH, HLRCC, LRCC, MCL, MCuL1, Fumarate hydratase Fumarase, Fumarate hydratase mitochondrial, MCuL 1, Multiple hereditary cutaneous leiomyomata., FMR0905-10 µg, FMR0905-20 µg, FMR0905-50 µg, FMR0905-100 µg, FMR0905-250 µg, FMR0905-500 µg, FMR0905-1 mg, FMR0905-10, FMR0905-20, FMR0905-50, FMR0905-100, FMR0905-250, FMR0905-500, FMR0905-1

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. 20 mM Tris-HCl buffer (pH 8.0)

Scientific Category

Cell Cycle

NCBI Accession Number

NP_000134

Uniprot Accession Number

P07954

Species

Human

AA 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

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