Aldolase B: Difference between revisions
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Aldolase B | {{DISPLAYTITLE:Aldolase B}} | ||
Aldolase B is an enzyme that plays a crucial role in the metabolism of fructose. It is one of the three aldolase | == Aldolase B == | ||
[[File:Aldolase_B_catalytic_mechanism.jpg|thumb|right|300px|Diagram of the catalytic mechanism of Aldolase B.]] | |||
'''Aldolase B''' is an enzyme that plays a crucial role in the [[metabolism]] of [[fructose]]. It is one of the three isoenzymes of [[aldolase]], the others being aldolase A and aldolase C. Aldolase B is primarily found in the [[liver]], [[kidney]], and [[intestine]]. | |||
==Function== | == Function == | ||
Aldolase B catalyzes the reversible cleavage of fructose-1-phosphate into dihydroxyacetone phosphate (DHAP) and glyceraldehyde. This reaction is a key step in the fructose metabolism | Aldolase B catalyzes the reversible cleavage of [[fructose-1,6-bisphosphate]] and [[fructose-1-phosphate]] into [[dihydroxyacetone phosphate]] (DHAP) and [[glyceraldehyde]]. This reaction is a key step in both [[glycolysis]] and [[gluconeogenesis]]. In the context of fructose metabolism, aldolase B is essential for the conversion of fructose into intermediates that can enter the glycolytic pathway. | ||
==Structure== | == Structure == | ||
Aldolase B is a homotetramer, meaning it is composed of four identical subunits. Each subunit contains a | Aldolase B is a homotetramer, meaning it is composed of four identical subunits. Each subunit contains a [[TIM barrel]] structure, which is a common motif in enzymes that catalyze reactions involving sugar phosphates. The active site of aldolase B contains a [[lysine]] residue that forms a [[Schiff base]] with the substrate, facilitating the cleavage of the carbon-carbon bond. | ||
== | == Clinical significance == | ||
Deficiency of aldolase B leads to a condition known as [[hereditary fructose intolerance]] (HFI). This is an [[autosomal recessive]] disorder characterized by the accumulation of fructose-1-phosphate in the liver, leading to [[hypoglycemia]], [[jaundice]], and [[hepatomegaly]]. Patients with HFI must avoid dietary fructose, sucrose, and sorbitol to prevent symptoms. | |||
== Related pages == | |||
==Related | |||
* [[Fructose metabolism]] | * [[Fructose metabolism]] | ||
* [[Glycolysis]] | * [[Glycolysis]] | ||
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[[Category:Enzymes]] | [[Category:Enzymes]] | ||
[[Category:Metabolism]] | [[Category:Metabolism]] | ||
[[Category: | [[Category:Genetic disorders]] | ||
Latest revision as of 05:40, 16 February 2025
Aldolase B[edit]
Aldolase B is an enzyme that plays a crucial role in the metabolism of fructose. It is one of the three isoenzymes of aldolase, the others being aldolase A and aldolase C. Aldolase B is primarily found in the liver, kidney, and intestine.
Function[edit]
Aldolase B catalyzes the reversible cleavage of fructose-1,6-bisphosphate and fructose-1-phosphate into dihydroxyacetone phosphate (DHAP) and glyceraldehyde. This reaction is a key step in both glycolysis and gluconeogenesis. In the context of fructose metabolism, aldolase B is essential for the conversion of fructose into intermediates that can enter the glycolytic pathway.
Structure[edit]
Aldolase B is a homotetramer, meaning it is composed of four identical subunits. Each subunit contains a TIM barrel structure, which is a common motif in enzymes that catalyze reactions involving sugar phosphates. The active site of aldolase B contains a lysine residue that forms a Schiff base with the substrate, facilitating the cleavage of the carbon-carbon bond.
Clinical significance[edit]
Deficiency of aldolase B leads to a condition known as hereditary fructose intolerance (HFI). This is an autosomal recessive disorder characterized by the accumulation of fructose-1-phosphate in the liver, leading to hypoglycemia, jaundice, and hepatomegaly. Patients with HFI must avoid dietary fructose, sucrose, and sorbitol to prevent symptoms.
