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F1757

Sigma-Aldrich

Fumarase from porcine heart

ammonium sulfate suspension, ≥300 units/mg protein (biuret)

Synonym(s):

L-Malate hydrolyase, Fumarate hydratase

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About This Item

CAS Number:
Enzyme Commission number:
EC Number:
MDL number:
UNSPSC Code:
12352204
NACRES:
NA.54

form

ammonium sulfate suspension

specific activity

≥300 units/mg protein (biuret)

foreign activity

malic dehydrogenase ≤0.1%

storage temp.

2-8°C

General description

Fumarase (FH in human) is a well-known tricarboxylic-acid-cycle enzyme found in both the cytoplasm and mitochondria of all eukaryotes.
Fumarase is mapped to human chromosome 1q43. Fumarase comprises of three domains D1, D2 and D3 and belongs to class II fumarase family.

Application

Fumarase from porcine heart has been used:
  • as a positive control in isocitrate dehydrogenase and α-ketoglutarate dehydrogenase assay
  • for immobilized enzyme based stability assay
  • in fumarate hydratase assays in human lung carcinoma cells (A549)

Fumarase is used as a protein calibration standard in the purification of intact DNA polymerase a/primase from mouse cells.

Biochem/physiol Actions

Fumarase catalyzes the reversible hydration of fumarate to malate. In its mitochondrial form, fumarate is involved in the Krebs Cycle, while the cytosolic form is involved in amino acid metabolism.
Fumarase is essential for regulation of cellular metabolism. Fumarase elicits protective function from double-strand breaks (DSBs) induced by infrared radiation. Loss of function mutations in the fumarase gene is implicated in hereditary leiomyomatosis and renal cell cancer (HLRCC)-syndrome.

Unit Definition

One unit will convert 1.0 μmole of L-malate to fumarate per min at pH 7.6 at 25 °C.

Physical form

Suspension in 3.2 M (NH4)2SO4, 0.05 M KH2PO4, pH 7.5, 0.014 M 2-mercaptoethanol

Preparation Note

Isolated by the method of Beeckmans, S., and Kanarek, L., Eur. J. Biochem., 78, 437 (1977). Affinity purified.

Pictograms

Exclamation mark

Signal Word

Warning

Hazard Statements

Hazard Classifications

Skin Sens. 1

Storage Class Code

10 - Combustible liquids

WGK

WGK 1

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable


Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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Fumarase Deficiency: A Safe and Potentially Disease Modifying Effect of High Fat/Low Carbohydrate Diet
Ryder B, et al.
JIMD Reports, 40(3), 77?83-77?83 (2018)
Fumarase: a paradigm of dual targeting and dual localized functions
Yogev O, et al.
FEBS Journal, 278(22), 4230-4242 (2011)
Study of an enzyme membrane reactor with immobilized fumarase for production of L-malic acid
Giorno L, et al.
Biotechnology and Bioengineering, 72(1), 77-84 (2001)
Fumarase-deficient Uterine Leiomyomas: An Immunohistochemical, Molecular Genetic, and Clinicopathologic Study of 86 Cases.
Miettinen M, et al.
American Journal of Surgical Pathology, 40(12), 1661-1669 (2016)
Fumarase: a mitochondrial metabolic enzyme and a cytosolic/nuclear component of the DNA damage response
Yogev O, et al.
PLoS Biology, 8(3), e1000328-e1000328 (2010)

Articles

Instructions for working with enzymes supplied as ammonium sulfate suspensions

Protocols

Enzymatic Assay of Fumarase

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