What is Maltase?
The maltase enzyme has the sole purpose of splitting maltose (modified starch) into separate glucose molecules (simple sugars) for digestion.
What is Maltose?
Maltose is a disaccharide or complex sugar that is made of two glucose molecules bonded together and is the second stage of the breaking down and digestion of starch into glucose sugars. The first stage is where amylase secreted by the pancreas and the salivary glands breaks starch down into maltose then maltases take over and complete the splitting of the molecules.
In humans and other mammals, maltase is found in the mucous membranes of the intestinal walls. They can also be found in plants, yeast and bacteria.
Acid Maltase Deficiency
Acid maltase deficiency (AMD) also known as glycogen storage disease type II (GSD II) also called Pompe (a Dutch pathologist) Disease is a degenerative genetically inherited disorder that keeps the muscles from being able to release the glycogen to be used by the body for energy causing a buildup in the muscles. Glycogen is the starch that has been unused during the digestion process and stored in the liver and muscles.
What is Acid Maltase?
Also known as acid alpha-glucosidase is the chemical or enzyme that is responsible for the regulation of the amounts of glycogen stored in the muscles of humans. When excessive amounts of glycogen are detected, the body will trigger the production of acid maltase to chemically transform the glycogen into readily available energy.
Acid Maltase Deficiency Symptoms
Some signs of acid maltase deficiency (AMD) are progressive weakness of the respiratory and mobile muscles leading to symptoms such as: heart and liver failure, weakness in the limbs (arms, legs, hands and feet) “floppy baby syndrome” (irregular muscle movement) in infants and flattening in muscle tone.
Acid Maltase Deficiency Treatment
Special diets have been shown to be effective such as those high in branched-chain amino acid proteins. Enzyme replacement therapy (ERT) is used widely for the treatment of Pompe disease as well.