Fumarase

Function

Fumarase is used in the citric acid cycle to conduct a transition step in the production of energy to make NADH. It metabolizes fumarate in the cytosol, which becomes a byproduct of the urea cycle and amino acid catabolism. It catalyzes the addition of water to make S-malate. This is a reversible reaction^[1]. See also:

• Krebs cycle carbons
• Krebs cycle importance
• Krebs cycle overview
• Krebs cycle step 7

Other interesting information

Fumarase or fumarate hydratase is dominant in fetal and adult tissues and largely expressed in the skin, parathyroid, lymph, and colon There are two classes of fumarases, which depend on the arrangement of their relative subunit, their metal requirement, and their thermal stability. Class I fumarases can change their state or become inactive when exposed to heat or radiation. They are sensitive to superoxide anions and Fe2+ dependent. Class II fumarases are found in eukaryotes and prokaryotes. They are iron-independent and thermal-stable.

Disease

Fumarase deficiency is an autosomal recessive metabolic disorder distinguished by a deficiency of the enzyme fumarate hydratase and indicated by an excess of fumaric acid in the urine. It is common in infants with neurologic abnormalities and its potential causes include cytosolic and mitochondrial forms of fumarase.

Fumarase Scenes