What is maltase? Maltase is an enzyme that catalyzes the hydrolysis of the disaccharide maltose to the simple sugar glucose. This enzyme is found in plants, bacteria, and yeast. Then there is what is called Acid maltase deficiency. It is categorized into three separate types based on the age of onset of symptoms in the affected individual.
Type a, or infantile, acid maltase deficiency usually begins to produce observable symptoms in affected individuals between the ages of two and five months.
Type b, or childhood, acid maltase deficiency usually begins to produce observable symptoms in affected individuals in early childhood. This type generally progresses much more slowly than infantile acid maltase deficiency.
Type c, or adult, acid maltase deficiency generally begins to produce observable symptoms in affected individuals in the third or fourth decades of life. This type progresses even more slowly than childhood acid maltase deficiency.
In humans and other vertebrates it is thought to be synthesized by cells of the mucous membrane lining the intestinal wall. During digestion, starch is partially transformed into maltose by the pancreatic or salivary enzymes called amylases; maltase secreted by the intestine then converts maltose into glucose. The glucose so produced is either utilized by the body or stored in the liver as glycogen (animal starch).
Maltase works at breaking down grain
For example, the sugar maltose is made from two glucose molecules bonded together. The enzyme maltase is shaped in such a way that it can break the bond and free the two glucose pieces. The only thing maltase can do is break maltose molecules, but it can do that very rapidly and efficiently. Other types of enzymes can put atoms and molecules together. Breaking molecules apart and putting molecules together is what enzymes do, and there is a specific enzyme for each chemical reaction needed to make the cell work properly.
Acid maltase deficiency (AMD), also known as Pompe disease, is a genetically inherited disease that affects muscle function. Inherited diseases are passed on from parents to a child. Patients with AMD have a defect, or mutation, in a gene that functions in muscles, called the acid alpha-glucosidase (GAA) gene. This genetic mutation causes a substance called glycogen to build up in the muscles of patients with AMD. Glycogen is a form of starch that is used to store short-term energy.
Acid maltase deficiency is also known as glycogen storage disease type II (GSD II) because it is characterized by a buildup of glycogen in the muscle cells. Glycogen is the chemical substance muscles use to store sugars and starches for later use.
Some of the sugars and starches from the diet that are not immediately put to use are converted into glycogen and then stored in the muscle cells. These stores of glycogen are then broken down into sugars, as the muscles require them.
Acid maltase is the chemical substance that regulates the amount of glycogen stored in muscle cells. When too much glycogen begins to accumulate in a muscle cell, acid maltase is released to break down this excess glycogen into products that will be either reabsorbed for later use in other cells or passed out of the body via the digestive system.
Individuals affected with acid maltase deficiency have either a complete inability or a severely limited ability to produce acid maltase. Since these individuals cannot produce the amounts of acid maltase required to process excess glycogen in the muscle cells, the muscle cells become overrun with glycogen. This excess glycogen in the muscle cells causes a progressive degeneration of the muscle tissues.
Glycogen buildup in the muscles of patients with AMD causes damage to the muscles, which leads to a progressive weakening of the muscles. This glycogen buildup may weaken the muscles of the heart and respiratory system. Cardiac or respiratory failure is the most common cause of death in patients with AMD.
AMD is estimated to affect about one in 40,000 to 100,000 births. The time of onset of AMD can vary among patients and can occur in infants, children, or adults. The infant onset form is considered to be the most severe, and usually results in death within one year. In general, the later the onset of AMD, the more slowly it will progress, and the less severe the symptoms. Children and adults diagnosed with AMD usually have a life expectancy from 10 to 20 years after symptoms begin to appear.
Symptoms Acid maltase deficiency- slowly progressive weakness of respiratory muscles and muscles of the hips, upper legs, shoulders and upper arms; cardiac involvement common in infantile-onset form
Progression Acid maltase deficiency – slowly progressive and less severe in childhood- and adult-onset forms; infantile form usually leads to death within first year of life unless treated early
In April 2006, the U.S. Food and Drug Administration approved Myozyme, an enzyme replacement therapy, for the treatment of acid maltase deficiency. This therapy has significantly improved the prognosis for patients.
Inheritance – autosomal recessive, meaning caused by the contribution of a defective gene from each parent.
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