The enzyme Fumarate Hydratase has a complex spelling that can be difficult to pronounce. In IPA phonetic transcription, it is /fjuːməˌreɪt haɪˈdreɪtəs/. The first syllable, "fju", is pronounced like "few." The "mə" sound is similar to "muh", and "reɪt" is "rate" with emphasis on the second syllable. "Haɪˈdreɪtəs" is pronounced "high-dray-tuhs." Fumarate Hydratase is important for energy production in the body and its correct spelling is crucial in scientific research.
Fumarate Hydratase, also known as FH, is an enzyme that plays a crucial role in the citric acid cycle, or the Krebs cycle, which is a key metabolic pathway occurring in the mitochondria of eukaryotic cells. This enzyme catalyzes the conversion of fumarate into malate by adding a molecule of water. It is an essential step in the cycle as it produces malate, which can then be further metabolized to generate energy in the form of ATP.
Fumarate Hydratase is a member of the lyase family of enzymes and is found in many organisms, including plants, animals, and bacteria. It is typically found in the cytoplasm of cells, although it can also be present within the mitochondria in certain tissues.
Mutations in the gene that encodes Fumarate Hydratase can lead to a rare genetic disorder called hereditary leiomyomatosis and renal cell cancer (HLRCC). This disorder is characterized by the development of benign tumors in smooth muscle tissue (leiomyomas) and an increased risk of certain types of kidney cancer. The loss of Fumarate Hydratase function results in the accumulation of fumarate, leading to alterations in cellular metabolism and promoting tumor growth.
Understanding the mechanism and function of Fumarate Hydratase is important not only for elucidating the complex intricacies of cellular metabolism but also for investigating potential therapeutic targets for certain cancers associated with its malfunction.
The word "Fumarate Hydratase" originates from the combination of two components: "fumarate" and "hydratase".
1. Fumarate: Fumarate is derived from the Latin word "fūmāre", which means "to smoke". It refers to fumaric acid, a chemical compound found in various plants and fruits.
2. Hydratase: "Hydratase" is a suffix commonly used to describe enzymes involved in the hydration of specific compounds. It derives from the term "hydrate", which means combining a substance with water.
When these two components are combined, "Fumarate Hydratase" refers to an enzyme involved in the hydration of fumarate, a process crucial in various biochemical pathways.