OGDH: Difference between revisions

Latest revision as of 20:20, 30 December 2024

OGDH

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[edit]

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[edit]

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[edit]

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.

Regulation[edit]

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.

References[edit]

External links[edit]

OGDH on WikiMD

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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 ==
-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 ==
-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 ==
-* [[Krebs cycle]]
+* [[Citric acid cycle]]
-* [[Enzyme]]
+* [[Mitochondrial diseases]]
-* [[Mitochondria]]
+* [[Enzyme regulation]]
-* [[Metabolic disorders]]
 == References ==
-<references />
+<references/>
+== External links ==
+* [https://www.wikimd.com/wiki/OGDH OGDH on WikiMD]
+{{Enzymes}}
+{{Citric acid cycle}}
 [[Category:Enzymes]]
-[[Category:Metabolism]]
+[[Category:Citric acid cycle]]
-[[Category:Genetic disorders]]
+[[Category:EC 1.2.4]]
-{{medicine-stub}}
+[[Category:Mitochondrial proteins]]