Fructose-bisphosphate aldolase
Fructose-bisphosphate aldolase, also known as aldolase, is an enzyme crucial in the process of glycolysis; it catalyzes the splitting of fructose 1,6-bisphosphate into glyceraldehyde 3-phosphate and dihydroxyacetone phosphate. This enzyme plays a pivotal role in the sugar metabolism pathway, facilitating the energy production process in cells. There are three isoforms of aldolase: A, B, and C, each encoded by a different gene and expressed in various tissues.
Function[edit | edit source]
Aldolase is a key enzyme in the glycolytic pathway, a metabolic pathway that converts glucose into pyruvate, releasing energy and producing adenosine triphosphate (ATP). Specifically, aldolase catalyzes the fourth step of glycolysis, the cleavage of fructose 1,6-bisphosphate into two three-carbon sugars, which can be further metabolized to generate energy. This reaction is reversible, allowing aldolase to also play a role in gluconeogenesis, the process of glucose synthesis from non-carbohydrate sources.
Isoforms[edit | edit source]
Aldolase A[edit | edit source]
Found predominantly in muscle tissue, aldolase A is involved in the glycolytic pathway, facilitating energy production during muscle contraction.
Aldolase B[edit | edit source]
Expressed mainly in the liver, kidney, and intestine, aldolase B is essential for fructose metabolism. Mutations in the gene encoding aldolase B are responsible for hereditary fructose intolerance (HFI), a condition that results in the inability to break down fructose, leading to serious metabolic disturbances.
Aldolase C[edit | edit source]
Aldolase C is primarily located in the brain and nervous system. Its exact function is less understood, but it is believed to play a role in the specific energy demands of the nervous system.
Structure[edit | edit source]
Aldolase is a tetramer composed of either four identical subunits (in the case of aldolase A and C) or a combination of subunits (in aldolase B). The enzyme's active site, where the catalysis occurs, binds fructose 1,6-bisphosphate. The structure of aldolase is critical for its function, as alterations can significantly affect its enzymatic activity.
Clinical Significance[edit | edit source]
Aldolase levels in the blood are sometimes measured as part of a diagnostic evaluation for muscle damage or disease. Elevated levels can indicate muscle damage or a neuromuscular disorder. Additionally, as mentioned, mutations in the aldolase B gene lead to hereditary fructose intolerance, a potentially life-threatening condition if untreated. Understanding the function and structure of aldolase can aid in the development of treatments for conditions related to this enzyme.
See Also[edit | edit source]
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Contributors: Prab R. Tumpati, MD