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	~~'''Fumarase''' (or '''fumarate hydratase''') is an~~ enzyme ~~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]] (also known as~~ the citric acid cycle ~~or the tricarboxylic acid cycle), which is central to cellular respiration. The cytosolic isoenzyme is involved in the metabolism of [[amino acids]] and [[fumarate]].~~		{{Short description\|An enzyme involved in the citric acid cycle}}
			{{Enzyme}}

	== ~~Function~~ ==		==Overview==
			'''Fumarase''', also known as '''fumarate hydratase''', is an enzyme that plays a crucial role in the [[citric acid cycle]], which is a key metabolic pathway that provides energy in the form of [[adenosine triphosphate]] (ATP) through the oxidation of [[acetyl-CoA]] derived from carbohydrates, fats, and proteins. Fumarase catalyzes the reversible hydration of [[fumarate]] to [[malate]].

	Fumarase ~~catalyzes~~ the ~~chemical reaction that transforms~~ fumarate ~~into~~ malate. The ~~reaction~~ is ~~as follows:~~		==Structure==
			Fumarase is a homotetrameric enzyme, meaning it is composed of four identical subunits. Each subunit contains an active site where the conversion of fumarate to malate occurs. The enzyme is highly conserved across different species, indicating its essential role in cellular metabolism.

	~~fumarate + H2O <~~=~~> malate~~		==Function==
			Fumarase functions in the mitochondria of eukaryotic cells, where it participates in the citric acid cycle. The reaction it catalyzes is:

	~~This reaction is a key step in the Krebs cycle, a series of chemical reactions used by all aerobic organisms to generate energy.~~		: Fumarate + H_O _ L-malate

	~~== Clinical significance ==~~		This reaction is important for the continuation of the citric acid cycle, which ultimately leads to the production of ATP, the energy currency of the cell. In addition to its role in energy production, fumarase is also involved in the regulation of [[reactive oxygen species]] and [[apoptosis]].

	Mutations in the fumarase ~~gene are associated with~~ [[fumarase deficiency]]~~, a rare autosomal recessive disorder~~ characterized by severe neurological impairment~~. Symptoms of fumarase deficiency may include~~ developmental delay~~, severe mental retardation~~, and ~~dysmorphic facial features~~.		==Clinical Significance==
			Mutations in the gene encoding fumarase can lead to a rare metabolic disorder known as [[fumarase deficiency]]. This condition is characterized by severe neurological impairment, developmental delay, and encephalopathy. Fumarase deficiency is inherited in an [[autosomal recessive]] pattern.

	~~In addition~~, ~~mutations in the fumarase gene have been associated with~~ [[hereditary leiomyomatosis and renal cell ~~cancer~~]] (HLRCC), ~~a disorder that increases the risk~~ of ~~developing benign tumors in~~ the ~~skin and uterus (leiomyomas) and a type~~ of ~~kidney cancer called renal cell carcinoma~~.		Fumarase has also been implicated in certain types of [[cancer]], particularly [[hereditary leiomyomatosis]] and [[renal cell carcinoma]] (HLRCC). In these cases, loss of fumarase activity leads to the accumulation of fumarate, which can act as an oncometabolite, promoting tumorigenesis.

	== ~~See also~~ ==		==Biochemical Pathway==
			[[File:Citric acid cycle with aconitate 2.svg\|thumb\|right\|300px\|Diagram of the citric acid cycle, showing the position of fumarase.]]
			Fumarase is a key enzyme in the citric acid cycle, which is depicted in the diagram. It catalyzes the conversion of fumarate to malate, which is then oxidized to [[oxaloacetate]] by [[malate dehydrogenase]]. This step is crucial for the regeneration of oxaloacetate, allowing the cycle to continue.

	* [[~~Krebs~~ cycle]]		==Research and Applications==
			Research into fumarase has provided insights into its role in cellular metabolism and its potential as a target for therapeutic intervention in metabolic disorders and cancer. Studies are ongoing to better understand the regulation of fumarase activity and its interactions with other metabolic pathways.

			==Related pages==
			* [[Citric acid cycle]]
	* [[Fumarate]]		* [[Fumarate]]
	* [[Malate]]		* [[Malate]]
	* [[~~Fumarase deficiency~~]]		* [[Mitochondria]]
	* [[~~Hereditary leiomyomatosis and renal cell cancer~~]]		* [[Metabolic pathway]]

	~~== References ==~~

	~~<references />~~

	~~{{stub}}~~

	[[Category:Enzymes]]		[[Category:Enzymes]]
	[[Category:Metabolism]]		[[Category:Metabolism]]
	[[Category:~~Genetic disorders~~]]		[[Category:Citric acid cycle]]
	~~[[Category:Oncology]]~~
	~~<gallery>~~
	~~File:1fuo2.jpg\|Fumarase~~
	~~File:GeneStructure.png\|Gene structure of Fumarase~~
	~~File:FumaraseMechanismE1CB.png\|Fumarase mechanism E1CB~~
	~~File:Reaction1.png\|Fumarase reaction~~
	~~</gallery>~~

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	[[Category:Genetic disorders]]		[[Category:Genetic disorders]]
	[[Category:Oncology]]		[[Category:Oncology]]
			<gallery>
			File:1fuo2.jpg\|Fumarase
			File:GeneStructure.png\|Gene structure of Fumarase
			File:FumaraseMechanismE1CB.png\|Fumarase mechanism E1CB
			File:Reaction1.png\|Fumarase reaction
			</gallery>

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2024-02-23T07:31:05Z

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'''Fumarase''' (or '''fumarate hydratase''') is an enzyme 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]] (also known as the citric acid cycle or the tricarboxylic acid cycle), which is central to cellular respiration. The cytosolic isoenzyme is involved in the metabolism of [[amino acids]] and [[fumarate]].

== Function ==

Fumarase catalyzes the chemical reaction that transforms fumarate into malate. The reaction is as follows:

fumarate + H2O <=> malate

This reaction is a key step in the Krebs cycle, a series of chemical reactions used by all aerobic organisms to generate energy.

== Clinical significance ==

Mutations in the fumarase gene are associated with [[fumarase deficiency]], a rare autosomal recessive disorder characterized by severe neurological impairment. Symptoms of fumarase deficiency may include developmental delay, severe mental retardation, and dysmorphic facial features.

In addition, mutations in the fumarase gene have been associated with [[hereditary leiomyomatosis and renal cell cancer]] (HLRCC), a disorder that increases the risk of developing benign tumors in the skin and uterus (leiomyomas) and a type of kidney cancer called renal cell carcinoma.

== See also ==

* [[Krebs cycle]]
* [[Fumarate]]
* [[Malate]]
* [[Fumarase deficiency]]
* [[Hereditary leiomyomatosis and renal cell cancer]]

== References ==

<references />

{{stub}}

[[Category:Enzymes]]
[[Category:Metabolism]]
[[Category:Genetic disorders]]
[[Category:Oncology]]

Fumarase - Revision history

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