Fructose-bisphosphatase 2

Fructose-bisphosphatase 2 (FBPase 2) is an enzyme that in humans is encoded by the FBP2 gene. This enzyme is a critical component of the gluconeogenesis pathway, which enables the body to produce glucose from non-carbohydrate sources.

Function

Fructose-bisphosphatase 2 is a liver enzyme that takes part in carbohydrate metabolism. It is one of the enzymes responsible for maintaining glucose homeostasis in the body. It catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate and inorganic phosphate, a key step in the gluconeogenesis pathway.

Structure

The FBPase 2 enzyme is a tetramer composed of four identical subunits. Each subunit has a molecular weight of approximately 37,000. The enzyme has two different conformations, depending on whether it is activated or inhibited.

Clinical significance

Mutations in the FBP2 gene are associated with fructose-1,6-bisphosphatase deficiency, a rare genetic disorder that results in episodic hypoglycemia, lactic acidosis, and ketosis.

See also

• Fructose 1,6-bisphosphatase
• Gluconeogenesis
• Fructose-1,6-bisphosphatase deficiency

References

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