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	'''OGDH''' or '''Oxoglutarate dehydrogenase''' is a key [[enzyme]] involved in the [[Krebs cycle]], also known as the citric acid cycle or TCA cycle. This enzyme catalyzes the conversion of alpha-ketoglutarate into succinyl-CoA and carbon dioxide, a critical step in the cycle that is responsible for the majority of cellular [[energy]] production.

	== ~~Structure~~ ==		{{Infobox enzyme
	OGDH is ~~a large, multi-subunit~~ enzyme ~~complex located~~ in the [[~~mitochondria~~]]. It ~~is composed~~ of ~~multiple copies of three different subunits: E1, E2,~~ and E3. ~~The E1 subunit~~ is a ~~thiamine pyrophosphate-dependent enzyme,~~ the ~~E2 subunit is a dihydrolipoamide succinyltransferase, and~~ the ~~E3 subunit is a dihydrolipoamide dehydrogenase~~.		\| name = 2-oxoglutarate dehydrogenase
			\| image = <!-- Image removed -->
			\| width =
			\| caption =
			\| EC_number = 1.2.4.2
			\| CAS_number = 9028-91-7
			\| GO_code = 0004591
			}}

			'''2-oxoglutarate dehydrogenase''' ('''OGDH''') is an important [[enzyme]] in the [[citric acid cycle]] (also known as the [[Krebs cycle]] or [[TCA cycle]]). It catalyzes the conversion of [[2-oxoglutarate]] (also known as [[alpha-ketoglutarate]]) into [[succinyl-CoA]] and [[carbon dioxide]]. This reaction is a key step in the metabolic pathway that generates [[adenosine triphosphate]] (ATP) through the oxidation of [[acetyl-CoA]].

	== Function ==		== Function ==
	~~The primary function of~~ OGDH is ~~to catalyze~~ the ~~decarboxylation of alpha~~-~~ketoglutarate~~, a ~~process~~ that ~~involves~~ the ~~removal~~ of ~~a carboxyl group. This reaction produces succinyl~~-~~CoA~~, a ~~compound that is then used~~ in the ~~next step of the Krebs~~ cycle. The ~~reaction also produces NADH~~, ~~a molecule~~ that ~~is used in the~~ [[~~electron transport chain~~]] ~~to produce ATP~~, ~~the main energy currency of the cell~~.		OGDH is a component of the [[2-oxoglutarate dehydrogenase complex]], which is a multi-enzyme complex that also includes [[dihydrolipoamide succinyltransferase]] and [[dihydrolipoamide dehydrogenase]]. This complex is responsible for the decarboxylation of 2-oxoglutarate, a critical step in the citric acid cycle.

			== Structure ==
			The enzyme is a large, multi-subunit complex that requires several [[cofactors]] for its activity, including [[thiamine pyrophosphate]] (TPP), [[lipoic acid]], [[coenzyme A]] (CoA), [[FAD]], and [[NAD+]].

	== Clinical significance ==		== Clinical significance ==
	Mutations in ~~the genes encoding the subunits of~~ OGDH can lead to ~~a variety of~~ [[~~metabolic disorders~~]]~~, including alpha-ketoglutarate dehydrogenase deficiency~~. ~~This rare disorder~~ is ~~characterized by a buildup of alpha-ketoglutarate and a decrease in the~~ levels ~~of succinyl-CoA~~ and ~~NADH. Symptoms~~ can ~~include developmental delay, seizures, and lactic acidosis~~.		Mutations or deficiencies in OGDH can lead to metabolic disorders and have been associated with [[neurological diseases]]. The enzyme's activity is crucial for maintaining normal cellular energy levels, and its dysfunction can result in [[mitochondrial diseases]].

	[[~~File:Alpha-ketoglutarate.svg\|thumb\|right\|Alpha~~-~~ketoglutarate, the substrate of OGDH~~]]		== Regulation ==
			OGDH activity is regulated by the availability of its substrates and cofactors, as well as by feedback inhibition from its products, such as [[NADH]] and [[succinyl-CoA]].

	== See also ==		== See also ==
	* [[~~Krebs~~ cycle]]		* [[Citric acid cycle]]
	* [[~~Enzyme~~]]		* [[Mitochondrial diseases]]
	* [[~~Mitochondria]]~~		* [[Enzyme regulation]]
	* [[Metabolic disorders]]

	== References ==		== References ==
	<references />		<references/>

			== External links ==
			* [https://www.wikimd.com/wiki/OGDH OGDH on WikiMD]

			{{Enzymes}}
			{{Citric acid cycle}}

	[[Category:Enzymes]]		[[Category:Enzymes]]
	[[Category:~~Metabolism~~]]		[[Category:Citric acid cycle]]
	[[Category:~~Genetic disorders~~]]		[[Category:EC 1.2.4]]
	~~{{medicine-stub}}~~		[[Category:Mitochondrial proteins]]

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'''OGDH''' or '''Oxoglutarate dehydrogenase''' is a key [[enzyme]] involved in the [[Krebs cycle]], also known as the citric acid cycle or TCA cycle. This enzyme catalyzes the conversion of alpha-ketoglutarate into succinyl-CoA and carbon dioxide, a critical step in the cycle that is responsible for the majority of cellular [[energy]] production.

== Structure ==
OGDH is a large, multi-subunit enzyme complex located in the [[mitochondria]]. It is composed of multiple copies of three different subunits: E1, E2, and E3. The E1 subunit is a thiamine pyrophosphate-dependent enzyme, the E2 subunit is a dihydrolipoamide succinyltransferase, and the E3 subunit is a dihydrolipoamide dehydrogenase.

== Function ==
The primary function of OGDH is to catalyze the decarboxylation of alpha-ketoglutarate, a process that involves the removal of a carboxyl group. This reaction produces succinyl-CoA, a compound that is then used in the next step of the Krebs cycle. The reaction also produces NADH, a molecule that is used in the [[electron transport chain]] to produce ATP, the main energy currency of the cell.

== Clinical significance ==
Mutations in the genes encoding the subunits of OGDH can lead to a variety of [[metabolic disorders]], including alpha-ketoglutarate dehydrogenase deficiency. This rare disorder is characterized by a buildup of alpha-ketoglutarate and a decrease in the levels of succinyl-CoA and NADH. Symptoms can include developmental delay, seizures, and lactic acidosis.

[[File:Alpha-ketoglutarate.svg|thumb|right|Alpha-ketoglutarate, the substrate of OGDH]]

== See also ==
* [[Krebs cycle]]
* [[Enzyme]]
* [[Mitochondria]]
* [[Metabolic disorders]]

== References ==
<references />

[[Category:Enzymes]]
[[Category:Metabolism]]
[[Category:Genetic disorders]]
{{medicine-stub}}

OGDH - Revision history

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