|
|
Fumarase
|
UniProt Number:
|
P07954
|
|
Alternate Names:
|
FH, fumarate hydratase, L-malate hydrolyase, S-malate hydrolyase
|
|
Structure and Function:
|
Fumarase catalyzes the reversible hydration/dehydration of fumarate to S-malate. Fumarase comes in two forms: mitochondrial and cytosolic. The mitochondrial form plays a role in the Citric Acid cycle and the cytosolic form is involved in the metabolism of amino acids and fumarate. Subcellular localization is established by the presence of a signal sequence on the amino terminus in the mitochondrial form.
|
|
Disease Associations:
|
Defects in FH are the cause of fumarase deficiency (FD) [MIM:606812] (also known as fumaricaciduria), multiple cutaneous and uterine leiomyomata (MCUL1) [MIM:150800], and hereditary leiomyomatosis and renal cell cancer (HLRCC) [MIM:605839].
|
Monoclonal Antibodies
|
Cat. No. |
Name |
Reactivity |
Apps. |
Amount |
|
MS709 |
Fumarase antibody |
human, bovine, rat |
ICC, IHC, IP, ICE, FLOW |
100 µg |
Enzyme Activity Assays
Protein Quantity Assays
|
|
Browse Products By:
|
|
|
|
|
|
Product Search:
|
|
|
Related Pathways:
Krebs Cycle
|
Subscribe to MitoNews
|
|
|
|
|
|
|
|
