By:
Who ever
Enzymes
Enzymes act as a catalyst by speeding up or slowing down processes in the body.
A protein enzyme has a particular shape that contains an active site.
An active site is where a substrate fits into place to have its bonds either built or broken.
At which time the products or product produced are released.
The enzyme remains to continue to act on any available substrate.
Hereditary Fructose Intolerance
AldolaseB is found mainly in the liver.
The second step in fructose metabolism is where aldolaseB breaks down fructose-1-phosphate into glyceraldehyde and dihydroxyacetone phosphate.
If there is a deficiency in aldolaseB, it slows down the metabolism of fructose allowing for a buildup in the liver.
A toxic build up of fructose in the liver can cause the death of liver cells.
Hereditary Fructose Intolerance
If fructose-1-phosphate is not being broken down by aldolaseB its products (glyceraldehyde and dihydroxyacetone phosphate) are not available to perform glycolysis in which the end product is ATP.
Also, the phosphate is bound to the Fructose-1-Phosphate and not available to the cells to produce ATP reducing the available ATP for the cells to use even more.
Also fructose-1-phosphate signals for glucokinase to stay in the cytoplasm of the cell, this does not allow for the liver to release glucose to help stabilize low blood sugars.
Fructose Metabolism
Fructose has a phosphate added to it by fructokinase into Fructose-1-Phosphate.
Fructose-1-phosphate is the substrate for aldolaseB where it is converted to glyceraldehyde and dihydroxyacetone phosphate.
Glyceraldehyde and dihydroxyacetone phosphate are used in glycolysis or the production of glycogen.
Lock and Key Model
Activation Energy
What would happen if the Cori Cycle only happened within a single cell.
The cycle would be unending repeating its self over and over, making it a futile cycle. Eventually using up the ATP available to the cell, because