Fumarase (or fumarate hydratase) is an enzyme (EC 4.2.1.2) that catalyzes the reversible hydration / dehydration of fumarate to malate. Fumarase comes in two forms: mitochondrial and cytosolic. The mitochondrial isoenzyme is involved in the Krebs cycle and the cytosolic isoenzyme is involved in the metabolism of amino acids and fumarate.

Fumarate hydratase (FH; also known as fumarase) is an enzyme found in both the cytoplasm and mitochondria of all eukaryotes. In mitochondria, FH is involved in generating energy for the cell through a metabolic pathway called the Krebs cycle. Its role in the cytoplasm, however, is unclear.

Fumarase is an enzyme of the tricarboxylic acid (TCA) cycle in mitochondria, but in recent years, it has emerged as a participant in the response to DNA double strand breaks (DSBs) in the nucleus.

Fumarase deficiency (or fumaric aciduria) is an exceedingly rare autosomal recessive metabolic disorder in the Krebs cycle, characterized by a deficiency of the enzyme fumarate hydratase, which causes a buildup of fumaric acid in the urine and a deficiency of malate.

Fumarase deficiency is a rare genetic disorder affecting the body's metabolism. This condition disrupts a specific enzyme responsible for energy production. It can lead to various health challenges.

Fumarase (EC 4.2.1.2) catalyzes the reversible dehydration of l-malate to fumaric acid. Fumarase plays a key role in the TCA cycle and is extensively distributed in microorganisms, plants, and animals. Fumarase can be divided into class I and class II with distinct properties summarized in Table 2. Much research has been carried out on the ...

Fumarase deficiency also called fumaric aciduria or fumarate hydratase deficiency, is an inherited condition that primarily affects the nervous system, especially the brain 1. Affected infants may have an abnormally small head size (microcephaly), abnormal brain structure, severe developmental delay, weak muscle tone (hypotonia), and failure to ...

Here we describe and discuss these fumarase related phenomena and in addition point out approaches for studying dual function of distributed proteins, in particular compartment-specific depletion.

Fumarate hydratase deficient (FH-d) uterine fibroids are a rare subtype that is diagnosed only on pathologic evaluation. FH-d uterine fibroids may be the first indicator of hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome.

Fumarase is an enzyme that catalyzes the reversible hydration of fumarate to malate in the citric acid cycle. This reaction is crucial for the continuation of the cycle, which plays a significant role in cellular respiration and energy production.